67740161 case-study-aml-intro

ANGELES UNIVERSITY FOUNDATION

GRADUATE SCHOOL

Master of Arts in Nursing

A Case Study On

ACUTE MYELOGENOUS LEUKEMIA

PRESENTED TO

ANNA LYN M.PAANO, RN, MAN

Professor, Concepts I

Presented By

EPIFANIA P. GOGOLIN, RN

MAN Student

I. INTRODUCTION

The terms malignant and cancerous describe cells that are growing and

proliferating in a disorderly, chaotic fashion. In adults, cancer usually occurs in the form

of a solid tumor. In children, the most frequent type of cancer is that of an immature cell

overgrowth, or leukemia (McCance & Heuther,2004).

Acute myelogenous leukemia (AML) is results from a defect from a

hematopoeitic stem cells that differentiates into all myeloid cells: monocytes,

granulocytes (neutrophils, basophils, eosinophils), erythrocytes and platelets (Smeltzer,

et al., 2004). AML comprises approximately 11% of the cases or leukemia in childhood

in the United States, with approximately 400 children diagnosed with AML annually.

Unlike ALL, the incidence of AML is constant from birth throughout the first 10

years of life with a slight peak in late adolescence. Internationally, AML is more

commonly diagnosed in developed countries. AML is also more common in men than in

women. There is no known relationship between race and the incidence of AML. It was

observed though, that AML is more common in whites than in other populations. The

difference is even more apparent in older patients. Prevalence increases with age.

However, this disease affects all age groups (Seiter, 2006).

The prognosis is highly variable. Patient age may be a factor; patients who are

younger may survive for 5 years or more after diagnosis of AML. However., patients who

are older or have a more undifferentiated form of AML tend to have worse prognosis.

Patients with supportive care usually survive less than 1 year, with death usually a result

of infection and hemorrhage (American Cancer Society, 2006).

AML can present with relative subtle, nonspecific symptoms such anorexia,

malaise, irritability, and weight loss. As the disease progresses, they present with signs

and symptoms associated with the failure of normal hematopoiesis, which results from

the replacement of normal marrow cells by malignant cells. The child may exhibit fatigue

pallor, and decreased activity due to anemia; easy bruising and bleeding associated with

thrombocytopenia; or fever and infection secondary to neutropenia. Bone pain and

arthralgias are less-common presenting symptoms in children with AML than those with

ALL. Massive hepatosplenomegaly is uncommon except in infants with AML (Seiter,

2006).

Bone marrow aspiration and biopsy are the definitive diagnostic tests.

Approximately 10% of patients with acute leukemia do not have circulating blasts at

diagnosis. One or more of the following lab tests may be used to diagnose AML and/or

to determine the specific subtype of AML. Samples of blood, bone marrow, or CSF are

looked at under a microscope by a pathologist and may be reviewed by the patient's

hematologist/oncologist. Based on the cells' size, shape, and other traits, doctors can

classify them into specific cell types. A key element of this classification is whether the

cells look like normal cells of circulating blood (mature) or lack features of normal blood

cells (immature).

The percentage of cells in the bone marrow or blood that are blasts (immature) is

particularly important. Having at least 20% blasts in the marrow or blood is generally

required for a diagnosis of acute myeloid leukemia. AML can also be diagnosed if the

blasts have a chromosome change that occurs only in a specific type of AML, even

though the blast percentage doesn't reach 20%. In order for a patient to be considered

to be in remission after treatment, the blast percentage must be no higher than 5%.

Additional tests that are used to confirm the diagnosis of AML are as follows.

Cytochemistry tests involve exposing cells to chemical stains (dyes) that react with only

some types of leukemia cells. Flow cytometry on the other hand is a technique which is

often used to look at the cells from bone marrow and blood samples. It is very accurate

in determining the exact type of leukemia. Related tests, called immunohistochemistry

tests, can be used to detect these substances when viewing cells under a microscope.

These tests are used for immunophenotyping--classifying leukemia cells according to

the substances (antigens) on their surfaces. There is also cytogenetics. These tests

examine a cell's chromosomes (long strands of DNA) under a microscope. Fluorescent

in situ hybridization (FISH) is a procedure similar to cytogenetic testing. It can find most

chromosome changes (such as translocations) that are visible under a microscope in

standard cytogenetic tests, as well as some changes too small to be seen with usual

cytogenetic testing. Very sensitive DNA tests such as polymerase chain reaction (PCR)

tests can also find translocations too small to be seen under a microscope, even if very

few leukemia cells are present in a sample. These tests may also be used after

treatment to find small numbers of leukemia cells that may not be visible under a

microscope.

There are also several imaging studies that might be done in people with AML,

but they are done more often to look for infections or other problems, rather than to look

for the leukemia itself. Because leukemia does not usually form visible tumors, imaging

tests are of limited value. In some cases imaging studies may be done to help

determine the extent of the disease, if it is thought it may have spread beyond the bone

marrow and blood. Routine chest x-rays may be done if a lung infection is suspected.

Computed tomography (CT) scan, Magnetic resonance imaging (MRI) scan, ultrasound,

GaLium amd bone scan can also be done. If leukemia is a possibility, a biopsy of the

area may be needed to confirm this.

The French-American-British (FAB) cooperative group has classified AML in to

eight subtypes (M0-M7) based on blast cell morphology and reactivity with histochemical

stains. A newer classification for hematopoietic and lymphoid neoplasms was developed

by the World Health Organization (WHO) that includes not only morphologic findings but

also genetic, immunophenotypic, biologic, and clinical features to define specific disease

entities. In the WHO classification the blast threshold for the diagnosis of AML is

reduced from 30 to 20% in the blood or marrow. In addition, patients with clonal

recurring cytogenetic abnormalities are classified as having AML regardless of blast

percentage.

The older, more traditional, FAB classification is as follows: M0 - Undifferentiated

leukemia;M1 - Myeloblastic without differentiation; M2 - Myeloblastic with differentiation;

M3 – Promyelocytic; M4 – Myelomonocytic; M4eo - Myelomonocytic with

eosinophilia;M5 - Monoblastic leukemia (M5a - Monoblastic without differentiation / M5b

- Monocytic with differentiation); M6 – Erythroleukemia; M7 - Megakaryoblastic leukemia

The newer WHO classification is as follows: 1) AML with recurrent genetic

abnormalities (AML with t(8;21)(q22;q22), (AML1/ETO) ; AML with abnormal bone

marrow eosinophils and inv(16)(p13q22) or t(16;16)(p13)(q22), (CBFB/MYH11) ; APL

with t(15;17)(q22;q12), (PML/RARa) and variants ; AML with 11q23 (MLL)

abnormalities): 2) AML with multilineage dysplasia (Following myelodysplastic syndrome

(MDS) or MDS/myeloproliferative disease (MPD); Without antecedent MDS or

MDS/MPD but with dysplasia in at least 50% of cells in 2 or more lineages): 3) AML and

MDS, therapy related ( Alkylating agent or radiation-related type; Topoisomerase II

inhibitor type; Others): 4) AML, not otherwise classified (AML, minimally differentiated;

AML, without maturation; AML, with maturation; Acute myelomonocytic leukemia; Acute

monoblastic or monocytic leukemia; Acute erythroid leukemia; Acute megakaryoblastic

leukemia; Acute basophilic leukemia; Acute panmyelosis and myelofibrosis; Myeloid

sarcoma).

Although several factors have been implicated in the causation of AML, most

patients who present with AML have no identifiable risk factor. Some congenital

disorders that predispose patients to AML include Bloom syndrome, Down syndrome,

congenital neutropenia, Fanconi anemia, and neurofibromatosis. Usually, these patients

develop AML during childhood; rarely, some may present in young adulthood. More

subtle genetic disorders, including polymorphisms of enzymes that metabolize

carcinogens, also predispose patients to AML. Several studies demonstrate a

relationship between radiation exposure and leukemia. Early radiologists (prior to

appropriate shielding) were found to have an increased likelihood of developing

leukemia. Persons who smoke have a small but statistically significant (odds ratio, 1.5)

increased risk of developing AML. In several studies, the risk of AML was slightly

increased in people who smoked compared with those who did not smoke. Exposure to

benzene is associated with aplastic anemia and pancytopenia. These patients often

develop AML.

Treatment of AML is divided into two stages: induction of remission and post

remission consolidation or intensification. Long-term maintenance treatment is no longer

generally used. All children should be referred to a pediatric oncology center for

treatment. Patients are placed into high-, intermediate- or low-risk groups, and their

chemotherapy protocols are adjusted accordingly. Approximately 50% of children treated

with chemotherapy alone appear to be cured of their leukemia. The outcome is

somewhat better for children who, while their first remission receive bone marrow

transplants from histocompatible sibling donors, with an 8-year survival rate of 60%. The

prognosis for children who do not enter remission or those that suffer from on-therapy

relapse is poor. Intensive reinduction therapies and bone marrow transplant for second

complete remission is associated with significant treatment-related morbidity and

mortality.

For current trends and issues, a number of new drugs are currently in clinical

trials. The two most promising areas are farnesyl transferase inhibitors and drugs that

target FLT3. Farnesyl transferase inhibitors target ras, a molecule that is involved in the

pathophysiology of various hematologic malignancies. Two drugs of this type, tipifarnib

and lonafarnib, are currently undergoing clinical trials. Early results have demonstrated

responses in 15-30% of patients with minimal toxicity. Drugs that target FLT3, such as

MLN518 and PKC412, are also currently being studied.

Newer treatment for AML is Gemtuzumab ozogamicin. Gemtuzumab ozogamicin

is a monoclonal antibody against CD33 (a molecule present on most AML cells but not

on normal stem cells) conjugated to calicheamicin (a potent chemotherapy molecule).

Gemtuzumab ozogamicin is currently approved by the Food and Drug Administration in

the United States for the treatment of patients with CD33-positive AML in first relapse

who are aged 60 years or older and who are not considered candidates for other

cytotoxic chemotherapy. Sievers reported the results of gemtuzumab ozogamicin

administration in 142 patients with AML who were in their first relapse and who had no

history of an antecedent hematologic disorder (AHD). Sixteen percent of patients

obtained a formal complete response. An additional 13% of patients met criteria for

complete response but did not have the required platelet recovery. Toxicity included

infusion reactions, myelosuppression, and hepatic toxicity. Newer studies are

investigating the use of gemtuzumab ozogamicin in combination with other

chemotherapy agents and in patients with newly diagnosed AML. Although gemtuzumab

ozogamicin is an active drug, the response rate is lower than that obtained with standard

"3 and 7" chemotherapy.

This particular case was chosen because it gave opportunity for the MAN student

to further learn and deepen her understanding about the condition and its treatment.

Aside from providing a learning experience, this case also allowed opportunity to

demonstrate the caring role that a nurse has.

OBJECTIVES

Patient-centered

After handling the patient and do proper nursing interventions, the patient with

the help of the significant others will be able to:

Short-term objectives:

1. Recognized the condition that are threatening to his health

2. Appreciate all the health teaching and cooperate with the nursing

interventions done for his comfort

3. Apply measures taught to prevent the worsening of his condition

Long-term objectives:

1. Maintain a healthy lifestyle that is contributory to health maintenance and

personal development

2. In time, sustain lifestyle that best ensure optimum level of functioning

3.Broaden knowledge regarding self-care measures at home

Nurse-centered

After handling patient and do proper nursing interventions, the nurse will

be able to:

Short-term objectives:

1. Establish a good nurse-client relationship with the client

2. Impart appropriate health teachings according to the clients needs

3. Describe illness and in-hospital experiences as they must appear to the child.

4. Established expected outcomes for the child.

5. Implement measures such as orientation, education and therapeutic play to

reduce stress of illness.

Long-term objective:

1. Improve/ deepen the nurses’ understanding, knowledge and skills with the

disease.

2. Promote effective nursing care to all the clients that may be handled in the

future with the same disease condition.

3. Apply/share the learning from the experience to colleagues, students, clients &

demonstrate a caring role as a nurse has.

4. Identify areas related to illness in children that could benefit from additional

nursing research or application of evidence-based practice.

II. Nursing Assessment

A. Personal History

The subject of this case study is given the name Totoy Bibo to protect his

real identity. He is a 3 year old, male, born on the 23rd of July year 2005 in one of

the medical hospital in Manila. A Filipino citizen, baptized as a Roman Catholic,

and a resident of 48 B Rizal St., Lapaz Tarlac. He was admitted in a tertiary

government hospital in Tarlac, last October 3, 2008 with chief complaints of

epistaxis, gum bleeding and low blood and platelet count.

The Bibo family is a nuclear type of family composing of three members;

his mother, father and Totoy Bibo himself. His father and mother are both 31

years of age. His father formerly works as a security guard at a firm in Manila and

is currently self-employed as a “balot & mani” vendor. His mother on the other

hand is a plain housewife. Mr. and Mrs. Bibo are not married and have been

living together for four years. This is Mrs. Bibo’s second family; she have five

children in her first husband. Totoy Bibo on the other hand is the only child with

her second husband.

The three of them are currently living in a compound comprising of

several families. According to Mrs. Bibo, their place is a wooden-concrete type of

building that is in bad condition because of a fire incident long ago. The residents

are also complaining of foul smelling air because of a poultry located infront of

their vicinity. Their toilet is water sealed and they have water pump as the source

of water supply. According to the mother their place is small but clean and well

maintained, with a small living room, one bedroom where the family sleeps

together, a kitchen and a comfort room. Their water is boiled before it is used by

the family. Mrs. Bibo also mentioned that the father of Totoy Bibo occasionally

smokes and drinks with his peers. The mother on the other hand is fond of

sweets and foods rich in carbohydrates, especially during her pregnancy. She

also admits that at an early age, they already offered Totoy Bibo softdrinks or

carbonated drinks for refreshment.

As for the families health belief and practices, the family believe in several

superstitions which affect their judgment and decisions sometimes. The Bibo

family believes in faith healers and “manghihilot” especially when someone get

fractures or sprains. They also make use of herbal medicines to heal several

conditions which they contemplate to be effective and at the same time

inexpensive. They also practice self-medications and more often than not buy

some over the counter drugs for common illnesses like paracetamol for fever.

They go to medical practitioners when they find no relief or the illness persistently

bothers them.

B. OBSTETRICAL HISTORY/ GROWTH & DEVELOPMENT

Mrs. Bibo’s last menstrual period was November 2004 and after nine months,

she delivered a healthy 8.9 lbs baby boy after five hours of labor at Quirino

Memorial Hospital in Manila, July 23, 2005 via normal spontaneous delivery.

During her nine months pregnancy, she recalls that she was fond of eating

watermelon and bananas. She cannot recall any serious illness or medications

taken. She claims that she did not have any problem at all during the pregnancy.

Totoy Bibo had his first bowel movement and urine output the first 24 hours

after delivery. His umbilical stump fell after 4 days. No jaundice, cyanosis, or

congenital anomalies were noted. Totoy Bibo have no known allergies for foods,

drugs, pollen or dust. Like her other children, Totoy Bibo was never breastfed by

his mother. He was first fed by formulated milk known as Bona, then after two

months was changed to rice milk or what is commonly called as “am” derived

from boiled rice. After that Totoy Bibo was fed in combination of Bear Brand, am

and Milo. He was than introduced to eating lugaw, mashed carrot, potato and

squash. It was also mentioned that even before reaching his first birthday, the

parents already give softdrinks for Totoy Bibo to drink. He is completely

immunized at the local health center in Lapaz, and was commonly noted to be

overweight during his monthly check ups.

Totoy Bibo being three years of age is a toddler under the Autonomy VS

shame and Doubt in Erik Erickson’s Psychosocial Development theory, where

the child focuses on self-control and confidence and failure to reinforce these

efforts will lead the child to doubt themselves and trust in them. As for his

cognitive development according to Jean Piaget, Totoy Bibo is under the Pre-

operational Thought where the thought of the child can comprehend simple

abstract and can arrive at answers mentally instead of physical attempts but

basically their thinking is still concrete and literal. Their type of play in this stage

is parallel play. As for Sigmund Freud’s PsychoAnalytical theory, Totoy bibo is in

the Anal Phase, where his interest is in self-discovery and finds pleasure and

sense of control in retention and defecation of feces & urine.

We can see Totoy Bibo’s autonomy by being able to feed himself and

verbalize and choose the food he wants to eat. The child is also clever and

understands the need for the oxygen cannula when he is having difficulty in

breathing. He also shows interest in playing with his room mates and he has a

robot and cars to play with. He has not been able to practice control in urination

and defection over some time because of the treatment and medication regimen

being imposed.

C. FAMILY-HEALTH ILLNES HISTORY

Legend:Red - Hypertension/Heart Disease Yellow – Tuberculosis

Green – Asthma

Figure 1. Genogram

The paternal grandparents of Totoy Bibo are known to have hypertensions and

all of his uncle and aunts including his father have asthma. In his maternal side, Totoy

Bibo’s grandfather have already passed away due to stroke. His grandmother on the

other hand is still living but has a history of tuberculosis. There are no known disease

or illness that have befallen her aunts and uncles in his mother side. All are in good

health as of the present.

D. HISTORY OF PAST ILLNESS

Totoy Bibo has a history of being brought at the emergency department of a

district hospital in Lapaz Tarlac several times at an early age of 1-2 years. The chief

complaints of which are mainly fever with convulsions and upper respiratory infections,

wherein her mother recalled that paracetamol, oxygen and nebulizations to be the

prompt treatment. Aside from this, there were no other illness or reasons for check up or

hospitalizations.

E. HISTORY OF PRESENT ILLNESS

Grandfather Grandmother Grandfather (†) Grandmother

Uncle 3

Uncle 2

Aunt 3Aunt 2Aunt Uncle 11

TOTOY BIBO

Uncle

Uncle

Aunt

Mother

Father

Uncle

March 29, 2008, Totoy Bibo was rushed to a tertiary government hospital in

Tarlac City after being suggested by the doctors in the district hospital in Lapaz when

results of his platelet and blood components were found to be below normal and in need

of immediate blood transfusion. Severe paleness and a marked decreased of

hemoglobin count of 20-25g/L was the chief complaint. He was then treated with packed

red blood cells and other unrecalled medication. After two weeks of treatment, Totoy

Bibo undergone bone marrow aspiration as ordered by his attending physician and was

diagnosed with Acute Myelogenic Leukemia-M1. Totoy Bibo then undergone

chemotherapy as advised, but the treatment was put to a halt after two months (2

cycles) because of financial constraints. For four months, he was in and out of the

hospital because of bleeding, paleness and severe dropped with his hemoglobin,

platelet and other blood components. Blood transfusions and other unrecalled

medications were given when Totoy Bibo was hospitalized. The persistence of epistaxis

and gum bleeding brought the patient back last October 3, 2008 and was then admitted.

F. PHYSICAL EXAMINATION

October 3, 2008, Emergency Room Pediatrics resident-on-duty’s assessment findings, as lifted from the chart

General: irritable & restless

Pale, palpebral conjunctiva, anicteric sclerae

With gum bleeding & epistaxis

With petechiae on both arms

Distended abdomen, no tenderness

Normal extremities

With vital signs of:

Temp : 37oC RR: 54 PR: 143

October 20, 2008 (Nurse-Patient Interaction )

General Survey

Seen patient on bed, with IVF of D5 0.3 NaCl at 250 cc level infusing on his left cephalic

vein. Patient is crying and restless; with body malaise; increased respiratory effort with

minimal movement; needs full assistance to do ADL. He was wearing a shirt and a pair

of pajamas. No appetite but with increased water intake (4-5 cups of water within an

hour). Vital signs taken as follows:

Temp: 37.4°C RR: 60HR: 154

Physical assessment:Head and Face:

a. Hair: with fine short black hairb. Scalp: no evidence of dandruff or flaking; no tenderness of the scalp

noted.c. Skull: normal skull configuration, no abnormal elevation or depression.d. Face: symmetrical in shape, no tenderness upon palpation

Eyes: a. General: eyes are symmetrical, with noted periorbital hematoma (racoon-

like eyes),b. Pupils: dark brown in color, with equal size approximately 2-3 mm,

positive PERRLAc. Eyebrows: equal distribution of hair on both sided. Eyelids and Eyelashes: no nodules and tenderness noted, with minimal

swelling of lower eyelid.e. Lacrimal puncta: no nodules and tenderness noted.f. Sclerae: whitish sclerae with evident capillaries, no jaundice noted.g. Conjunctiva: pale conjunctiva.h. Vision: with good visual acuity

Ears: a. External: symmetrical appearance of pinna, no lesions or abnormal

discharges or swelling.b. Internal: no discharges notedc. Cranial Nerve VIII: no hearing impairment of both ears.

Nose: Symmetrical nares, no discharge, exudates, no bleeding nor swelling

noted. Mouth and Throat:

a. Mouth/Lips: dry & cracked, with swelling & sores on both lips and gums with minimal bleeding noted.

b. Tongue: no lesion and ulceration and able to take tongue out when instructed

c. Soft palate and uvula: no swelling on palate and uvulad. Throat: no sore throat noted

Neck:a. General: no difficulty with neck mobility

b. Blood vessels: slight distension of jugular vein is noted.c. Trachea: no masses noted, and no thyroid gland enlargement.

Integumentary: a. Skin: pale in color, with warm skin, petechial rashes on both palm of the

hands, with minute hematoma noted on joint areas on both upper and lower extremity,

b. Nails: short and clean; with capillary refill of 3 seconds, absence of clubbing and discoloration

c. Hair: with equal distribution of hairLymph Nodes

With slightly enlarged cervical lymph nodesChest/Back:

a. Respiration: with notable increase in respiratory rate, with slight difficulty in breathing

b. Lungs: Rales auscultated from both lung fields.c. Chest: with equal chest expansion, with equal anterioposterior

diameter, no abnormal retraction, circumference measuring 48 cm in width. Visible heart beat noted

d. Back: with minute hematoma at the back & prominent at shoulder blades

Cardiovascular: a. Heart: positive S1 and S2 sounds, no murmurb. Peripheral pulses: negative palpitations with normal rate

Gastrointestinal:a. Abdomen: with rigid abdomen; abdominal girth of 62 cm; no striae or

rashes noted, no protruding mass or discharge on the umbilicus, no ascites, normoactive bowel sounds, no rebound tenderness, no tenderness on kidneys and liver upon palpation.

Musculoskeletal:a. Upper Extremities: equal in size, symmetrical in shape with no evident

distension of veins, and equal pulses, no edema noted. Pale with petechial rashes on both palms of the hands and minute hematoma on joint areas.

b. Lower Extremities: equal in size, symmetrical in shape with no evident distension of veins, and equal pulses. With enlarged bleeding hematoma at the left foot measuring about 4-5cm in circumference with wound dressing.

October 21, 2008 ( during the Nurse-Patient Interaction )

General Survey

Seen patient on bed, with IVF of D5 0.3 NaCl at 500 cc level infusing on his left cephalic

vein. Patient is crying and restless; with body malaise; increased respiratory effort with

minimal movement; needs moderate assistance to do ADL. He was wearing a shirt and

a pair of pajamas. With minimal appetite (Patient observed chewing hotdogs but not

swallowing the food). With increased water intake (4-5 1/2 cups of water within an hour).

Vital signs taken as follows:

Temp: 37.8°C RR: 44HR: 120



noted.c. Skull: normal skull configuration, no abnormal elevation or depression.d. Face: symmetrical in shape, no tenderness upon palpation and no

crepitus heard while moving the jawEyes:

a. General: eyes are symmetrical, with noted periorbital hematoma (racoon-like eyes)

b. Pupils: black in color, with equal size approximately 2-3 mm, positive PERRLA

c. Eyebrows: equal distribution of hair on both sided. Eyelids and Eyelashes: no evidence of infection, no nodules and

tenderness noted, with minimal swelling of lower eyelid.e. Lacrimal puncta: no nodules and tenderness noted.f. Sclerae: whitish sclerae with evident capillaries, no jaundice noted.g. Conjunctiva: pale conjunctiva.h. Vision: with good visual acuity

Ears: a. External: symmetrical appearance of pinna, no lesions or

abnormal discharges or swelling.b. Internal: no discharges notedc. Cranial Nerve VIII: no hearing impairment of both ears



a. Mouth/Lips: dry & cracked, with swelling & sores on both lips and gums with minimal bleeding noted.



Neck:a. General: no difficulty with neck mobilityb. Blood vessels: slight distension of jugular vein is noted.c. Trachea: no masses noted, and no thyroid gland enlargement.

Integumentary: a. Skin: pale in color, with warm skin, petechial rashes on both palm of

the hands, with minute hematoma noted on joint areas on both upper and lower extremity,





b. Lungs: Rales auscultated from both lung fields.c. Chest: with equal chest expansion, with equal anterioposterior




Gastrointestinal:a. Abdomen: with rigid abdomen; abdominal girth of 63 cm; no striae or

rashes noted, no protruding mass or discharge on the umbilicus, no ascites, normoactive bowel sounds, no rebound tenderness, no tenderness on kidneys and liver upon palpation.





General Survey

Seen patient sitting on bed, with IVF of D5 0.3 NaCl at 500 cc level infusing on his left

cephalic vein, interacts with other patient; with slight body malaise; increased respiratory

effort with minimal movement; needs minimal assistance to do ADL. He was wearing a

shirt and a pair of pajamas. With increased appetite (Patient observed drinking

chocolate drink, eating hotdogs biscuits, and sandwiches , swallowing them). Still with

increased water intake (4-5 1/2 cups of water within an hour). Vital signs taken as

follows:

Temp: 37.4°C RR: 44HR: 126





a. General: eyes are symmetrical, with noted hematoma periorbital edema (racoon-like eyes)

b. Pupils: black in color, with equal size approximately 2-3 mm, positive PERRLA

c. Eyebrows: equal distribution of hair on both sided. Eyelids and Eyelashes: no evidence of infection, no nodules and

tenderness noted, with minimal swelling of lower eyelid.e. Lacrimal puncta: no nodules and tenderness noted.f. Sclerae: whitish sclerae with evident capillaries, no jaundice noted.g. Conjunctiva: pale conjunctiva.h. Vision: with good visual acuity


discharges or swelling.b. Internal: no discharges notedc. Cranial Nerve VIII: no hearing impairment of both ears



a. Mouth/Lips: dry & cracked, with swelling & sores on both lips and gums with no bleeding noted.



Neck:a. General: no difficulty with neck mobilityb. Blood vessels: slight distension of jugular vein is noted.c. Trachea: no masses noted, and no thyroid gland enlargement.


the hands, with minute hematoma noted on joint areas on both upper and lower extremity





b. Lungs: Rales auscultated from both lung fieldsc. Chest: with equal chest expansion, with equal anterioposterior




Gastrointestinal:a. Abdomen: with abdominal distention, abdominal girth of 64 cm; no

striae or rashes noted, no protruding mass or discharge on the umbilicus, no ascites, normoactive bowel sounds, no rebound tenderness, no tenderness on kidneys and liver upon palpation.





General Survey

Seen on bed, with Oxygen inhalation at 5-6Lpm, with IVF of Plain NSS 1 liter at 900 cc

level infusing on his left cephalic vein, with ongoing 1 unit of PRBC S#5914 as side drip,

(+) pallor, restless and irritable, barely know the people handling him including his

parents, generalized body weakness; with exertional breathing.





a. General: eyes are symmetrical, with noted hematoma periorbital edema (racoon-like eyes)

b. Pupils: black in color, PERRLA (not assessed)c. Eyebrows: equal distribution of hair on both side

d. Eyelids and Eyelashes: no evidence of infection, no nodules and tenderness noted, with minimal swelling of lower eyelid.

e. Lacrimal puncta: no nodules and tenderness noted.f. Sclerae: whitish sclerae with evident capillaries, no jaundice noted.g. Conjunctiva: pale conjunctiva.h. Vision: with good visual acuity


discharges or swelling.b. Internal: no discharges notedc. Cranial Nerve VIII: no hearing impairment of both ears



a. Mouth/Lips: dry & cracked, with dried wound on both lips, with mild sores on oral cavity, with no bleeding noted.

b. Tongue: not assessedc. Soft palate and uvula: not assessedd. Throat: not assessed

Neck:a. General: no difficulty with neck mobilityb. Blood vessels: distended jugular vein.c. Trachea: no masses noted, and no thyroid gland enlargement.


the hands, with minute hematoma noted on joint areas on both upper and lower extremity

b. Nails: short and clean; with capillary refill of 5-6 secondsc. Hair: with equal distribution of hair

Lymph NodesWith slightly enlarged cervical lymph nodes

Chest/Back:a. Respiration: with notable increase in respiratory rate, with slight

difficulty in breathingb. Lungs: Rales auscultated from both lung fields.c. Chest: with equal chest expansion, with equal anterioposterior

diameter, with labored breathing, circumference measuring 48 cm in width. Visible heart beat noted



Gastrointestinal:a. Abdomen: with abdominal distention, abdominal girth of 72 cm

Musculoskeletal:

a. Upper Extremities: equal in size, symmetrical in shape with no evident distension of veins, and equal pulses, no edema noted. Pale with petechial rashes on both palms of the hands and minute hematoma on joint areas.

b. Lower Extremities: equal in size, symmetrical in shape with no evident distension of veins, and equal pulses. With enlarged bleeding hematoma at the left foot measuring about 6-7cm in circumference with wound dressing & elastic bandage.

III. ANATOMY & PHYSIOLOGY

The Bone Marrow

The Bone marrow is the soft, flexible, vascular tissue found in the hollow interior

cavities and cancellous bone spaces in the center of many bones and which is the

source of erythrocytes (red blood cells) and leukocytes (white blood cells).

There are two main types of bone marrow. Red bone marrow is the center of production

of all blood cells except one type of lymphocyte, which matures in the thymus. Yellow

bone marrow stores fats.

As the source of blood cells, the bone marrow is critical to the health of people. The

disruption of the intricate harmony, such as the production of too many, too few, or

abnormal blood cells, results in diseases, such as leukemia, that can be life-threatening.

http://www.newworldencyclopedia.org/entry/Fat

http://www.newworldencyclopedia.org/entry/Thymus

http://www.newworldencyclopedia.org/entry/Lymphocyte

http://www.newworldencyclopedia.org/entry/Leukocyte

http://www.newworldencyclopedia.org/entry/Erythrocyte

http://www.newworldencyclopedia.org/entry/Bone

http://www.newworldencyclopedia.org/entry/Biological_tissue

Medical procedures have been developed to examine the bone marrow (bone marrow

aspiration and biopsy) of patients and also to transfer normal stem cells from a donor

into a recipient (bone marrow transplantation).

STRUCTURE

Red marrow consists primarily of a loose, soft network of blood vessels and protein

fibers interspersed with developing blood cells. The blood vessels are termed the

vascular component, and the protein fibers and developing blood cells collectively are

referred to as the stroma, or the extravascular component. The protein fibers crisscross

the marrow, forming a meshwork that supports the developing blood cells clustered in

the spaces between the fibers.

Red marrow contains a rich blood supply. Arteries transport blood containing oxygen

and nutrients into the marrow, and veins remove blood containing carbon dioxide and

other wastes. The arteries and veins are connected by capillaries, blood vessels that

branch throughout the marrow. In various places, the capillaries balloon out, forming

numerous thin, blood-filled cavities. These cavities are called sinusoids, and they assist

in blood-cell production.

Yellow marrow is so named because it is composed of yellow fat cells interspersed in a

rich mesh of connective tissue that also supports many blood vessels. While not usually

actively involved in blood formation, in an emergency yellow marrow is replaced by

blood-forming red marrow when the body needs more blood.

Gray's Anatomy illustration of cells in bone marrow. (From New World Encyclopedia)

FUNCTIONS

Red marrow produces all of the body’s blood cells—red blood cells, white blood cells,

and platelets. Red blood cells in the circulatory system transport oxygen to body tissues

and carbon dioxide away from tissues. White blood cells are critical for fighting bacteria

and other foreign invaders of the body. Platelets are essential for the formation of blood

clots to heal wounds.

Within red bone marrow, all blood cells originate from a single type of cell, called a

hematopoietic stem cell. Stimulated by hormones and growth factors, these stem cells

divide to produce immature, or progenitor blood cells. Most of these progenitor cells

remain in the stroma and rapidly undergo a series of cell divisions, producing either red

blood cells or white blood cells. At any one time, the stroma consists largely of

progenitor cells in various stages of development. At the appropriate developmental

stage, the fresh, new cells squeeze through the walls of the capillaries. From there, the

cells leave the bone and enter the body’s circulatory system. Some progenitor cells

http://encarta.msn.com/encyclopedia_761566878/Circulatory_System.html

migrate to the sinusoids, where they produce platelets, which also travel to the

circulatory system via the capillaries.

Although stem cells are relatively rare—about 1 in every 10,000 marrow cells is a stem

cell—they typically produce the forerunners of an estimated 2 million red cells per

second and 2 billion platelets per day. However, if significant amounts of blood are lost

or other conditions reduce the supply of oxygen to tissues, the kidneys secrete the

hormone erythropoietin. This hormone stimulates stem cells to produce more red blood

cells. To fight off infection, hormones collectively termed colony stimulating growth

factors are released by the immune system. These hormones stimulate the stem cells to

produce more infection-fighting white blood cells. And in severe cases, the body

converts yellow marrow into red marrow to help produce needed blood cells.

THE HEMATOPOIETIC SYSTEM

Hematology is the science of blood and blood forming tissues. It includes both

cellular and non-cellular blood components. Hematologic activities occur in many organs

of the body and have the potential for multiple forms of pathology. Blood itself is

composed of two elements – the liquid component, plasma, and the solid components,

which are mainly erythrocytes, leukocytes, and thrombocytes. These elements are

formed by hematopoiesis.

Hematopoiesis is the continuous, regulated formation of blood cells. There are

three primary functions of hematopoiesis.

1. Oxygen delivery

2. Hemostasis

3. Host defense

Note that some complexity is omitted from the diagram. Lymphocytes come from

"Lymphoid" line, whereas granulocytes, monocytes, megakaryocytes, and erythrocytes

come from "Myeloid" line. Among myeloid cells, granulocytes and monocytes have a

common precursor, "CFU-GM".

Hematopoiesis occurs in the bone marrow. The degree and location of bone

marrow activity varies depending on the age and health status of your patient. Within the

bone marrow there is a pluripotent stem cell. This stem cell is the “Mother Cell” or the

originator of all blood cells. It has the ability to self-renew and create progenitor stem cell

lines. They are naturally limited in number. By reviewing the chart above, you can see

that all cells come from the stem cell. An attack on the stem cell can theoretically affect

all of them similarly. A disease or agent that impacts erythroblasts could impact all the

cell type in that “line,” but not those in a different “line.”

The Blood

http://en.wikipedia.org/wiki/Monocytes

http://en.wikipedia.org/wiki/Granulocytes

http://en.wikipedia.org/wiki/Erythrocytes

http://en.wikipedia.org/wiki/Megakaryocytes

http://en.wikipedia.org/wiki/Monocytes

http://en.wikipedia.org/wiki/Granulocytes

http://en.wikipedia.org/wiki/Lymphocytes

http://en.wikipedia.org/wiki/File:Illu_blood_cell_lineage.jpg

Blood is a liquid tissue. Suspended in the watery plasma are seven types of cells

and cell fragments. The Red Blood Cells (RBCs) or erythrocytes , Platelets or

thrombocytes, and five kinds of white blood cells (WBCs) or leukocytes. Three kinds of

granulocytes are as follows: Neutrophils, Eosinophils, Basophils. Two kinds of

leukocytes without granules in their cytoplasm are: Lymphocytes and Monocytes,

Functions of the BloodBlood performs two major functions: transport through the body of : oxygen and

carbon dioxide, food molecules (glucose, lipids, amino acids), ions (e.g., Na+, Ca2+,

HCO3−), wastes (e.g., urea), hormones, heat and defense of the body against infections

and other foreign materials. All the WBCs participate in these defenses.

Red Blood Cells (erythrocytes)

The most numerous type in the blood. Women average about 4.8 million of these

cells per cubic millimeter (mm3; which is the same as a microliter [µl]) of blood. Men

average about 5.4 x 106 per µl. These values can vary over quite a range depending on

such factors as health and altitude. (Peruvians living at 18,000 feet may have as many

as 8.3 x 106 RBCs per µl.) RBC precursors mature in the bone marrow closely attached

to a macrophage. They manufacture hemoglobin until it accounts for some 90% of the

dry weight of the cell. The nucleus is squeezed out of the cell and is ingested by the

macrophage. No-longer-needed proteins are expelled from the cell in vesicles called

exosomes. Thus RBCs are terminally differentiated; that is, they can never divide. They

live about 120 days and then are ingested by phagocytic cells in the liver and spleen.

Most of the iron in their hemoglobin is reclaimed for reuse. The remainder of the heme

portion of the molecule is degraded into bile pigments and excreted by the liver. Some 3

million RBCs die and are scavenged by the liver each second. Red blood cells are

responsible for the transport of oxygen and carbon dioxide.

Hemoglobin

Hemoglobin is a protein that is carried by red cells. It picks up oxygen in the lungs

and delivers it to the peripheral tissues to maintain the viability of cells. Hemoglobin is

made from two similar proteins that "stick together". Both proteins must be present for

the hemoglobin to pick up and release oxygen normally. One of the component proteins

is called alpha, the other is beta. Before birth, the beta protein is not expressed. A

hemoglobin protein found only during fetal development, called gamma, substitutes up

until birth.

In adult humans the hemoglobin (Hb) molecule consists of four polypeptides: two

alpha (α) chains of 141 amino acids and two beta (β) chains of 146 amino acid. Each of

these is attached the prosthetic group heme. There is one atom of iron at the center of

each heme. One molecule of oxygen can bind to each heme.

Like all proteins, the "blueprint" for hemoglobin exists in DNA (the material that

makes up genes). Normally, an individual has four genes that code for the alpha protein,

or alpha chain. Two other genes code for the beta chain. (Two additional genes code for

the gamma chain in the fetus). The alpha chain and the beta chain are made in precisely

equal amounts, despite the differing number of genes. The protein chains join in

developing red blood cells, and remain together for the life of the red cell.

Hemoglobin synthesis requires the coordinated production of heme and globin.

Heme is the prosthetic group that mediates reversible binding of oxygen by hemoglobin.

Globin is the protein that surrounds and protects the heme molecule.

Erythrocytes and Related Values

Red Blood Cell (RBC)Normal Range: 4.6-6.3 X106/mm3 (males)4.2 -5.4 X106/mm3 (females)

Erythrocytes, or red blood cells, originate from a stem cell. Vitamin B12, folic acid, iron,

and copper are essential in the formation of erythrocytes. Erythropoietin is released by

kidneys in response to hypoxemia which stimulates the bone marrow to produce red

blood cells. Typically, red blood cells live approximately 120 days. When the red blood

cells become old and damaged, the liver, spleen, and bone marrow cleanse them from

the blood.

Reticulocyte CountNormal Range: 0.5-2.5% of RBCsWhen released from the bone marrow red blood cells are slightly immature and are

known as reticulocytes. Reticulocytes mature into red blood cells within a few days.

HemoglobinNormal Range: 14-18 g/dL (males)12-16 g/dl (females)Hemoglobin is a protein-iron compound in red blood cell that carries oxygen. This

laboratory value is used to evaluate the oxygen-carrying capacity of the blood. Red

blood cells and hemoglobin go hand in hand. One unit of packed red blood cells

generally equals one whole number increase in your hemoglobin value. For example:

If your patient’s hemoglobin is 7.0 g/dl, and you give him one unit of packed red blood

cells, your patient’s hemoglobin should come up to 8.0 g/dl.

HematocritNormal Range: 42-52% (males)37-57% (females)Hematocrit is an expression of the total percentage of blood volume that is composed of

red blood cells. It is also known as the packed cell volume of your blood (Sherwood,

1997).

IronNormal Range: 50-150 mcg/dLAs mentioned earlier, iron is necessary for the formation of hemoglobin, an essential part

of the red blood cell. Iron is absorbed from the small intestine into the blood and binds

with transferrin. Transferrin transports iron tothe bone marrow where it is used to make

hemoglobin.

Total Iron Binding CapacityNormal Range: 250-410 mcg/dlThe amount of iron that can still bind with transferrin (to be transported to bone marrow

to make hemoglobin) is known as the total iron binding capacity or TIBC. Think of your

TIBC as the total amount of people that can get on a bus. The iron is the people and the

bus is transferrin. When your serum iron levels increase, your TIBC decreases. When

you serum iron levels decrease, then your TIBC increases.

FerritinNormal Range: 20 - 300 ng/mL (males)20 - 120 ng/mL (females)Ferritin is a protein that binds to iron. Most of the iron stored in the body is attached to

ferritin. Ferritin is found in the liver, spleen, and bone marrow. Only a small amount is

found in the blood. Like the TIBC, the amount of ferritin in the blood may help indicate

the amount of iron stored in your body.

White Blood Cell Count (WBC) and Differential

White blood cells, or leukocytes, are classified into two main groups:

granulocytes and nongranulocytes (also known as agranulocytes). The granulocytes,

which include neutrophils, eosinophils, and basophils, have granules in their cell

cytoplasm. Neutrophils, eosinophils, and basophils also have a multilobed nucleus. As a

http://www.rnceus.com/cbc/cbcdiff.html

result they are also called polymorphonuclear leukocytes or "polys." The nuclei of

neutrophils also appear to be segmented, so they may also be called segmented

neutrophils or "segs." The nongranuloctye white blood cells, lymphocytes and

monocytes, do not have granules and have nonlobular nuclei. They are sometimes

referred to as mononuclear leukocytes.

The lifespan of white blood cells ranges from 13 to 20 days, after which time they

are destroyed in the lymphatic system. When immature WBCs are first released from the

bone marrow into the peripheral blood, they are called "bands" or "stabs." Leukocytes

fight infection through a process known as phagocytosis. During phagocytosis, the

leukocytes surround and destroy foreign organisms. White blood cells also produce,

transport, and distribute antibodies as part of the body's immune response.

LeukocytesTotal WBCNormal Range: 5,000 -10,000/microliter

Leukocytes, or white blood cells, protect the body from bacteria and infection.

The white blood cell count is expressedas the number of leukocytes per microliter of

blood. The total WBC count increases in response to infection or trauma.

LymphocytesNormal Range: 16-46%

There are several kinds of lymphocytes (although they all look alike under the

microscope), each with different functions to perform . The most common types of

lymphocytes are B Lymphocytes ("B cells"). These are responsible for making

antibodies. T lymphocytes ("T cells"). There are several subsets of these: Inflammatory

T-cells that recruit macrophages and neutrophils to the site of infection or other tissue

damage. Cytotoxic T-Lymphocytes (CTLs) that kill virus-infected and, perhaps, tumor

cells. Helper T-cells that enhance the production of antibodies by B cells.

Although bone marrow is the ultimate source of lymphocytes, the lymphocytes

that will become T cells migrate from the bone marrow to the thymus where they mature.

Both B cells and T cells also take up residence in lymph nodes, the spleen and other

tissues where they encounter antigens; continue to divide by mitosis; mature into fully

functional cells. Lymphocytes mature in the lymph nodes. They live approximately 100-

300 days. The total lymphocyte count represents total T and B lymphocytes. T

lymphocytes are killer cells. They tell B lymphocytes to make antibodies. Lymphocytes

increase in viral illnesses, such as measles, mumps, chicken pox, influenza, viral

hepatitis, mononucleosis, and in acute transplant rejection.

MonocytesNormal Range: 0-12%

Monocytes leave the blood and become macrophages. Macrophages are large,

phagocytic cells that engulf foreign material (antigens) that enter the body dead and

dying cells of the body. They ingest cellular debris at the area of infection or

inflammation. They increase after several days of active infection or inflammation. They

are like your body’s garbage truck: they are a little slow, but they pick up all the

“garbage” or cellular debris and take it away.

NeutrophilsNormal Range: 40-70%

The most abundant of the WBCs. This photomicrograph shows a single

neutrophil surrounded by red blood cells. Neutrophils squeeze through the capillary walls

and into infected tissue where they kill the invaders (e.g., bacteria) and then engulf the

remnants by phagocytosis. This is a never-ending task, even in healthy people: Our

throat, nasal passages, and colon harbor vast numbers of bacteria. Most of these are

commensals, and do us no harm. But that is because neutrophils keep them in check.

However, heavy doses of radiation, chemotherapy, and many other forms of stress can

reduce the numbers of neutrophils so that formerly harmless bacteria begin to

proliferate. The resulting opportunistic infection can be life-threatening. Leukocyte types

are counted and expressed as a percentage. Neutrophils are the predominant type of

granulocytes. Neutrophils are special soldiers in your body’s immunity army. Their main

responsibility is to kill bacteria, destroy bacteria’s ability to reproduce, and destroy

bacteria’s ability to produce endotoxins.

BandsNormal Range: 0-8%

Neutrophil’s primal cell type is bands. Bands are adolescent neutrophils. It is

abnormal to have elevated bands in your blood stream. When the percent of bands is

increased you have a “shift to the left.” Historically, the diagram of the hematopoietic

system was read from left to right, not top to bottom as it does today. Thus, if you had an

increase in a cell type, moving left to the progenitor cell – you would have a shift to the

left.

EosinophilsNormal Range: 0-7%

The number of eosinophils in the blood is normally quite low (0–450/µl).

However, their numbers increase sharply in certain diseases, especially infections by

parasitic worms. Eosinophils are cytotoxic, releasing the contents of their granules on

the invader. Eosinophils are responsible for fighting parasites, and are increased in

allergic or autoimmune disorders. For example, eosinophils increase when a patient has

hives due to allergic reaction.

BasophilsNormal Range: 0-1%

The number of basophils also increases during infection. Basophils leave the

blood and accumulate at the site of infection or other inflammation. There they discharge

the contents of their granules, releasing a variety of mediators such as: histamine,

serotonin, prostaglandina and leukotrienes which increase the blood flow to the area and

in other ways add to the inflammatory process. The mediators released by basophils

also play an important part in some allergic responses such as hay fever and an

anaphylactic response to insect stings. Histamine and heparin and increase only in the

healing process.

Leukocytosis, a WBC above 10,000, is usually due to an increase in one of the

five types of white blood cells and is given the name of the cell that shows the primary

increase. Neutrophilic leukocytosis = neutrophilia, Lymphocytic leukocytosis =

lymphocytosis, Eosinophilic leukocytosis = eosinophilia, Monocytic leukocytosis =

monocytosis, Basophilic leukocytosis = basophilia.

PhysiologyIn response to an acute infection, trauma, or inflammation, white blood cells

release a substance called colony-stimulating factor (CSF). CSF stimulates the bone

marrow to increase white blood cell production. In a person with normally functioning

bone marrow, the numbers of white blood cells can double within hours if needed. An

increase in the number of circulating leukocytes is rarely due to an increase in all five

types of leukocytes. When this occurs, it is most often due to dehydration and

hemoconcentration. In some diseases, such as measles, pertussis and sepsis, the

increase in white blood cells is so dramatic that the picture resembles leukemia.

Leukemoid reaction, leukocytosis of a temporary nature, must be differentiated from

leukemia, where the leukocytosis is both permanent and progressive.

Therapy with steroids modifies the leukocytosis response. When corticosteroids

are given to healthy persons, the WBC count rises. However, when corticosteroids are

given to a person with a severe infection, the infection can spread significantly without

producing an expected WBC rise. An important concept to remember is that,

leukocytosis as a sign of infection can be masked in a patient taking corticosteroids.

Leukopenia occurs when the WBC falls below 4,000. Viral infections,

overwhelming bacterial infections, and bone marrow disorders can all cause leukopenia.

Patients with severe leukopenia should be protected from anything that interrupts skin

integrity, placing them at risk for an infection that they do not have enough white blood

cells to fight. For example, leukopenic patients should not have intramuscular injections,

rectal temperatures or enemas.

Leukocytes: critical low and high values

A WBC of less than 500 places the patient at risk for a fatal infection. A WBC

over 30,000 indicates massive infection or a serious disease such as leukemia. When a

patient is receiving chemotherapy that suppresses bone marrow production of

leukocytes, the point at which the count is lowest is referred to as the nadir.

Blood ClottingPlateletsNormal Range: 150,000 – 400,000/microliter

Platelets are cell fragments produced from megakaryocytes. Blood normally

contains 150,000–350,000 per microliter (µl) or cubic millimeter (mm3). This number is

normally maintained by a homeostatic (negative-feedback) mechanism. If this value

should drop much below 50,000/µl, there is a danger of uncontrolled bleeding because

of the essential role that platelets have in blood clotting. Some causes: certain drugs and

herbal remedies; autoimmunity.

When blood vessels are cut or damaged, the loss of blood from the system must

be stopped before shock and possible death occur. This is accomplished by solidification

of the blood, a process called coagulation or clotting. A blood clot consists of a plug of

platelets enmeshed in a network of insoluble fibrin molecules. Platelets are small,

colorless cells that have a lifespan of seven to ten days. They perform three major roles:

1) decreasing the luminal size of damaged vessels to decrease blood loss, 2) forming

blockages in injured vessels to decrease blood loss, and 3) with plasma providing the

correct ingredients needed to accelerate blood coagulation.

THE CLOTTING CASCADEThe end result of the clotting cascade is fibrin clots, fibrin, and thrombin. When

the clotting cascade is activated, usually due to vessel injury or damage, platelets are

one of the first responders. They stick to the damaged vessel and recruit more platelets

to the site. This aggregation of platelets forms a temporary plug that safeguards the

vessel wall from further bleeding. Simultaneously, additional proteins from the clotting

cascade are activated in a specific order that lead to the formation of fibrin. Fibrin is a

very sticky substance and acts as glue at the site, securing the platelet plug. Finally, the

clot must be dissolved in order for normal blood flow to resume following tissue repair.

The dissolution of the clot occurs through the action of plasmin. Plasmin is a protein that

is responsible for digesting fibrin. Eventually, scar tissue forms completing the healing of

the injured vessel (Sherwood, 1997).

PlasmaPlasma is a straw-colored, clear liquid that is ninety percent water. It is essential

for the transport of our blood components. Besides water, plasma also contains

dissolved electrolytes responsible for membrane excitability, plasma proteins that

maintain the osmotic distribution of fluid and substances capable of buffering pH

changes (Sherwood, 1997). Plasma transports materials needed by cells and materials

that must be removed from cells: various ions (Na+, Ca2+, HCO3−, etc.; glucose and

traces of other sugars; amino acids; other organic acids; cholesterol and other lipids;

hormones; urea and other wastes. Most of these materials are in transit from a place

where they are added to the blood (a "source") exchange organs like the intestine and

depots of materials like the liver to places ("sinks") where they will be removed from the

blood, every cell and exchange organs like the kidney, and skin.

IV. THE PATIENT AND HIS ILLNESS

A.PATHOPHYSIOLOGY (BOOK CENTERED)

Predisposing Factors: Race ( White race )Gender (Male)Age (increases with age)Heredity/Familial Tendency

Affectations in different committed cells

Mutant leukemia cells proliferate and fill the bone marrow

Compete and infiltrate hematopoeisis &

Precipitating Factors:Antecedent HematologicDisordersCongenital DisorderEnvironmental Exposures (high doses of radiation, chemicals like benzene, tobacco Smoke)Prior Exposure To Chemotherapeutic Agents For Another Malignancy

Disruption of pluripotent stem cells

Disruption of specific genes

Bone Pain

PetechiaeEcchymosis

Gingival bleedingepistaxis

Bleeding Tendencies

pallorAnemia

Wt. loss Malaise

Easy fatigability

Erythroblasts

Proliferation of immature

phagocytes

Decreased production of normal RBC

Megakaryoid Committed Cells

Megakaryoblast


megakaryocytes

Myeloid Committed Cells

Myeloblasts


myelocytes

Lymphoblasts


lymphocytes

Affectations of B lymphocytes

& T-lymphocytes

Erythroid Committed Cells

Risk for infection


Monoblasts


monocytes

Lymphoid Committed Cells

Affectations in WBC cells components

NeutrophilsBasophils

Eosinophils

Inability to protect body against

invasion

Leukoblast

a. SYNTHESIS OF THE DISEASE

GENERAL DESCRIPTION

The underlying pathophysiology consists of a maturational arrest of bone marrow

cells in the earliest stages of development. The mechanism of this arrest is under study,

but in many cases, it involves the activation of abnormal genes through chromosomal

translocations and other genetic abnormalities. This developmental arrest results in 2

disease processes. First, the production of normal blood cells markedly decreases,

which results in varying degrees of anemia, thrombocytopenia, and neutropenia.

Second, the rapid proliferation of these cells, along with a reduction in their ability to

undergo programmed cell death (apoptosis), results in their accumulation in the bone

marrow, blood, and, frequently, the spleen and liver.

b. RISK FACTORS

PRE-DISPOSING FACTORS:

RACE - AML is more common in whites than in other populations.

SEX - AML is more common in men than in women. The difference is even more

apparent in older patients. Some have proposed that the increased prevalence of AML in

men may be related to occupational exposures.

AGE - Prevalence increases with age. The median age of onset is 65 years. However, this disease affects all age groups.

FAMILIAL TENDENCY - Germ-line mutations in the gene AML1 (RUNX1, CBFA2) occur

in the familial platelet disorder with predisposition for AML, an autosomal-dominant

disorder characterized by moderate thrombocytopenia, a defect in platelet function, and

propensity to develop AML. Some hereditary cancer syndromes, such as Li-Fraumeni

syndrome, can manifest as leukemia.

PRECIPITATING FACTORS:

ANTECEDENT HEMATOLOGIC DISORDERS - Unknown etiology that occurs most

often in older patients and manifests as progressive cytopenias that occur over months

to years. Other that predispose patients to AML include aplastic anemia, myelofibrosis,

paroxysmal nocturnal hemoglobinuria, and polycythemia vera.

CONGENITAL DISORDERS - Some congenital disorders that predispose patients to

AML include Bloom syndrome, Down syndrome, congenital neutropenia, Fanconi

anemia, and neurofibromatosis. More subtle genetic disorders, including polymorphisms

of enzymes that metabolize carcinogens, also predispose patients to AML.

ENVIRONMENTAL EXPOSURES -Several studies demonstrate a relationship between

radiation exposure and leukemia. Early radiologists (prior to appropriate shielding) were

found to have an increased likelihood of developing leukemia. Patients receiving

therapeutic irradiation for ankylosing spondylitis were at increased risk of leukemia.

Survivors of the atomic bomb explosions in Japan were at a markedly increased risk for

the development of leukemia. Persons who smoke have a small but statistically

significant (odds ratio, 1.5) increased risk of developing AML. In several studies, the risk

of AML was slightly increased in people who smoked compared with those who did not

smoke. Exposure to benzene is associated with aplastic anemia and pancytopenia.

These patients often develop AML. Many of these patients demonstrate M6 morphology.

PRIOR EXPOSURE TO CHEMOTHERAPEUTIC AGENTS FOR ANOTHER

MALIGNANCY - As more patients with cancer survive their primary malignancy and

more patients receive intensive chemotherapy (including bone marrow transplantation

[BMT]), the number of patients with AML increases because of exposure to

chemotherapeutic agents. Patients with a prior exposure to alkylating agents, with or

without radiation, often have a myelodysplastic phase prior to the development of AML.

The typical latency period between drug exposure and acute leukemia is approximately

3-5 years for alkylating agents/radiation exposure but only 9-12 months for

topoisomerase inhibitors.

C. SIGNS AND SYMPTOMS WITH RATIONALE

1. Anemia, Neutropenia, and Thrombocytopenia – These are due to bone marrow

failure. It results from the fact that as leukemic clone of cells grows, it tends to displace

development of normal blood cells in the bone marrow. There is also decreased

neutrophil levels despite an increased total WBC count.

2. Physical signs of anemia, including pallor and a cardiac flow murmur, are frequently

present – These are due to the increased number of white blood cells displacing or

otherwise interfering with the production of normal blood cells in the bone marrow. The

most common symptom of anemia is fatigue. Patients often retrospectively note a

decreased energy level over past weeks. Other symptoms of anemia include dyspnea

upon exertion, dizziness, and, in patients with coronary artery disease, anginal chest

pain. Myocardial infarction may be the first presenting symptom of acute leukemia in an

older patient.

3.Fever and other signs of infection can occur, including lung findings of pneumonia –

These are due to the lack of normal white blood cell production that makes the patient

susceptible to infections, while the leukemic cells are derived from white blood cell

precursors, they have no infection-fighting capacity. Patients present with fever, which

may occur with or without specific documentation of an infection. Patients with the lowest

absolute neutrophil counts (ie, <500 cells/µL and especially <100 cells/µL) have the

highest risk of infection. Patients also often have a history of upper respiratory infection

symptoms that have not improved despite empiric treatment with oral antibiotics.

4. Abnormal Bleeding ( nosebleeds, gingival bleeding, purpura, ecchymosis, petechiae

–These are due to thrombocytopenia. Patients usually demonstrate petechiae,

particularly on the lower extremities. Petechiae are small, often punctate, hemorrhagic

rashes that are not palpable. Areas of dermal bleeding or bruises (ie, ecchymoses) that

are large or present in several areas may indicate a coexistent coagulation disorder such

as DIC. Purpura is characterized by flat bruises that are larger than petechiae but

smaller than ecchymoses. Potentially life-threatening sites of bleeding include the lungs,

gastrointestinal tract, and the central nervous system.

5. Signs relating to organ infiltration with leukemic cells – The most common sites

of infiltration include the spleen, liver, and gums. These include hepatosplenomegaly

and, to a lesser degree, lymphadenopathy Patients with splenomegaly note fullness in

the left upper quadrant and early satiety. . Occasionally, patients have skin rashes due

to infiltration of the skin with leukemic cells (leukemia cutis). Chloromata are

extramedullary deposits of leukemia. Rarely, a bony or soft-tissue chloroma (solid

leukenic mass or tumor outside of the bone marrow) may precede the development of

marrow infiltration by AML (granulocytic sarcoma).

6. Bone Pain - Patients with a high leukemic cell burden may present this symptom

which is caused by increased pressure in the bone marrow.

7. Signs relating to leukostasis - Patients with markedly elevated WBC counts

(>100,000 cells/µL) can present with symptoms of leukostasis (ie, respiratory distress

and altered mental status). Leukostasis is a medical emergency that requires immediate

intervention.

B. PATHOPHYSIOLOGY (CLIENT-CENTERED)

Predisposing Factors:

Gender (Male)Age (3 years old)

Mutant leukemia cells proliferate and fill the bone marrow

Compete and infiltrate hematopoeisis &

Disruption of pluripotent stem cells

Disruption of specific genes

Precipitating Factors:

Environmental Exposures- Cigarette Smoke- Exposure to certain chemicals (carbonated drinks even before reaching 1-yr of age/ foul-smelling env’t cause by nearby poultry)

• Petechiae* (both palms of the hand)

• Ecchymosis*

• Gingival bleeding*

• Epistaxis (upon admission)

• Hematoma* -Periorbital -in the sole of left foot measuring 6-7 cm diameter

Bleeding Tendencies


Erythroblasts


phagocytes

Decreased production of normal RBC

Megakaryoid Committed Cells

Megakaryoblast


megakaryocytes

Myeloid Committed Cells

Myeloblasts


myelocytes

Erythroid Committed Cells


Monoblasts


monocytes

Affectations in WBC cells

components

Neutrophils

Lymphocytes

Presence of Blast Cells

Inability to protect body

against invasion

(10-/7/8/14-19-08)

(10-7/8/12/14-19-08)

(10-7/8/12/14-19-08)

10- 3/7/8/12/14/15/17/18/19-08

INFECTION

Signs of infection

• On & off Fever

(10-3/12/13/17/18/19/25- 08)

• Acute

• Organ

Infiltration(distended abdo-

men)

• Leuko-Stasis

• Altered Mental Status

• R

a. SYNTHESIS OF THE DISEASE (CLIENT BASED)

GENERAL DESCRIPTION

The underlying pathophysiology consists of a maturational arrest of bone marrow

cells in the earliest stages of development. This developmental arrest results in 2

disease processes. First, the production of normal blood cells markedly decreases,

which results in varying degrees of anemia, thrombocytopenia, and neutropenia.

Second, the rapid proliferation of these cells, along with a reduction in their ability to

undergo programmed cell death (apoptosis), results in their accumulation in the bone

marrow, blood, and, frequently, the spleen and liver. In AML, the bone

b. RISK FACTORS

PRE-DISPOSING FACTORS:

SEX - AML is more common in men than in women. The difference is even more

apparent in older patients.

AGE- Prevalence increases with age. The median age of onset is 65 years. However,

this disease affects all age groups.

PRECIPITATING FACTORS:

ENVIRONMENTAL EXPOSURES - In several studies, the risk of AML was slightly

increased in people who smoked compared with those who did not smoke.

C. SIGNS AND SYMPTOMS WITH RATIONALE

Pallor*

Malaise/ Fatigue*

(10-7/8/12/14-19-08)

Dyspnea ( RR, PR)(10-3/4/5/6/16/18/25-08)

Anemia*

DEATH

1. Anemia, Neutropenia, and Thrombocytopenia – These are due to bone marrow

failure. It results from the fact that as leukemic clone of cells grows, it tends to displace

development of normal blood cells in the bone marrow. There is also decreased

neutrophil levels despite an increased total WBC count.

2. Physical signs of anemia- including pallor and dyspnea upon exertion.These are

due to the increased number of white blood cells displacing or otherwise interfering with

the production of normal blood cells in the bone marrow. The most common symptom of

anemia is fatigue.

3. Fever and other signs of infection Patients present with fever, which may occur

with or without specific documentation of an infection. These are due to the lack of

normal white blood cell production that makes the patient susceptible to infections, while

the leukemic cells are derived from white blood cell precursors, they have no infection-

fighting capacity.

4. Abnormal Bleeding ( nosebleeds, gingival bleeding, purpura, ecchymosis, petechiae

–These are due to thrombocytopenia.

5. Signs relating to organ infiltration with leukemic cells – The most common sites

of infiltration include the spleen, liver, and gums. These include hepatosplenomegaly

and, to a lesser degree, lymphadenopathy.

6. Bone Pain - Patients with a high leukemic cell burden may present this symptom

which is caused by increased pressure in the bone marrow.

7. Signs relating to leukostasis - Patients with markedly elevated WBC counts

(>100,000 cells/µL) can present with symptoms of leukostasis (ie, respiratory distress

and altered mental status). Leukostasis is a medical emergency that requires immediate

intervention.

67740161 case-study-aml-intro

Education

Transcript of 67740161 case-study-aml-intro