Hematology Alteration

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    Nursing management of patient with blood

    disorder

    objective:

    After four hours of lecture discussion, the level 1V student will be able to:Review the anatomy and physiology of the blood

    Relate the diagnostic test result to the blood disorder

    Discuss different blood disorder

    Develop nursing care plan utilizing nursing process in caring patient with blooddisorders

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    Anatomy and physiology of the blood

    Blood

    is an aqueous mixture consisting ofplasma and cells

    - Is a sticky opaque fluid with a metallic taste- Is slightly alkaline, pH of 7. 35 7. 45

    - Temp. of 38C or 100. 4 F

    - 8% of body weight, volume in healthy males is 5 6 liters

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    Plasma

    - is a straw colored liquid consisting of approximately 90% water and 10

    % protein

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    Characteristics and function of formedelements of the blood

    1. Erythrocytes (rbc)

    - 4 6 million per mm

    - Salmon colored biconcave disks, sacs of hgb. Most

    organelles have been ejected

    - Fxn: transport O2 bound to hgb molecules; also transport

    small amount of co2

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    2. Leukocytes (wbc)- 4000- 11, 000mm

    - Granulocytes:

    a. Neutrophils 40 70% of wbc; active phagocytes; number

    increases rapidly during short-term or acute infectionsb. Eosinophils 1- 4% of wbc; kills parasitic worms; increase

    during allergy attack; might phagocytize antigen-antibodycomplexes and inactivate some inflammatory chemicals

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    c. Basophils 0- 1% of wbc- Granules contain histamine ( vasodilator chemicals) which is

    discharge at sites of inflammation

    Agranolucytes:

    a. Lymphocytes-

    20 45 % of wbc- Part of immune system; one group (B lymphocytes) produces

    antibodies; other group (T lymphocytes) involved in graft rejection,fighting tumors and viruses, and activating B lymphocytes

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    b. Monocytes 4 8% 0f wbc

    -Active phagocytes that become macrophages in the tissues;

    long-term clean-up; increase in number during chronic

    infections such as TB

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    3. Platelets- 250,000- 500,000

    - Called megakaryocytes coz they are fragments of bizarre

    multinucleate

    - Needed for normal blood clotting; initiate clotting cascade by

    clinging to broken area; helps to control blood loose from

    broken blood vessels

    - Accelerated by the hormone thrombopoietin

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    How blood cell formulated?

    -through hematopoiesis that occurs in red bone marrow or myeloid tissue

    - In flat bones: skulls and pelvis, ribs, sternum and proximal epiphyses of thehumerus and femur

    - Each type of blood cells- is produced in different numbers in response tochanging body needs and different stimuli

    - Matured cells are discharge into the blood vessels surrounding the area

    - All the formed elements arise from a common types of stem cells thehemocytoblast in red bone marrow

    - The stem cells renews itself by mitosis

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    What regulate the RBC?

    - Erythropoietin circulates in the blood at all times and RBCS

    are formed in constant rate.

    - Kidney plays the major role in releasing this hormones, liverproduce some

    NORMAL LIFESPAN FOR AN RBC = 120 DAYS

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    hematocrit

    - Is the % of blood cells in a volume of blood

    - Normal value= 42% - 45% of blood volume

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    HEMOSTASIS

    - Stoppage of blood flow

    - This response which is fast and localized involves many

    substances normally present in plasma, as well as some thatare released by platelets and injured tissue cells.

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    PHASES OF HEMOSTASIS

    Platelet plug forms sticky and cling to the damage site, Anchoredplatelets release chemicals that attract more platelets to the site toformed a white thrombus or platelet plug.

    Vascular spasm occur anchored platelet release serotonin whichcause the blood vessel s into spasm resulting to decrease blood lossafter clotting can occur

    Coagulation event occur- release of thromboplastin. Coagulationtime = 3 to 6 minutes

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    BLOOD TYPES

    BLOOD GROUP - RBC ANTIGEN PLASMA ANTIBODIES- RECEIVED BLOOD

    AB--------------------------------A,B----------------------------------NONE---------------------------------------------A,B,AB,O

    B-------------------------------------B-------------------------------------ANTI A--------------------------------------------B,O

    A-----------------------------------A-------------------------------------ANTI B------------------------------------------A,O

    O------------------------------NONE-------------------------------ANTI A, B----------------------------------O, UNIVERSAL DONOR

    Antigen is a substance that body recognizes as foreign, it stimulates the immune

    system to release antibodies

    RH BLOOD GROUP the RBC carry the-Rh antigen

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    DISORDERS AFFECTINGTHE RBCS

    POLYCYTHEMIA Secondary

    - Increase RBC and Hgb production

    - Compensatory response to chronic hypoxia:Hypoxia ->increase Erythropoietin production by the kidneys-

    >stimulation of the bone marrow to increase RBC production

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    POLYCYTHEMIA VERA/primary

    Occurs most frequently in middle age and Jewish men

    Hyperplasia of the bone marrow

    Increase RBC (erythrocytosis); increaseWBC (leukocytosis);

    increase platelets (thrombocytosis)Pathophysiology:

    Increase rbc,wbc,platelet -> increase Bld.volume and Viscosity ->thromboembolism and capillary overdistention -> rupture - >hemorrhage ->hypovolemia; Organ infiltration -> hepatomegaly,Spleenomegaly,cerebral hypoxia,arthralgia

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    Etiology

    Unknown

    Predisposing factors:

    Long term hypoxia: COPD,CHF,SMOKING. LIVINGIN HIGHALTITUDE

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    Signs and Symptoms:

    Symptoms of two are the same

    Headache, epistaxis, dizziness, tinnitus, blurred vision, fatigue,weakness, pruritus, exertional dyspnea, increased BP and pulse

    Ruddy complexion, lips become reddish purple Petechia and bruises due to platelet dysfunction

    Susceptible for thrombi formation

    Causes hyperuricemia increase uric acid bld level

    RBC count is over 6 million; Hgb over 18mg/dl, Hct over 55%

    Increased risk for CVA and MI due to thromboembolism

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    Nursing management

    ASSESSMENT:

    History taking for the s&s

    Assess for nutritional status for inadequate dietary intake due to GIsymptoms of fullness and dyspepsia

    Observe for bruises and changes in color

    Check for neck vessels distention, listen apical, radial, pedal pulses

    Check Homans signs and edema

    Observe for dyspnea, epistaxis

    Listen to the breath sound

    Checked pupils responses and presence of numbness or tingling

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    Nursing diagnosis

    Ineffective Tissue Perfusion r/t decreased blood circulation

    Knowledge deficit r/t disease process and treatment

    Risk for injury r/t dizziness

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    IRON DEFICIENCY ENEMIA:

    - most common type of anemia and occurs when the body

    does not have enough iron to synthesize functional hgb.

    - Found frequently in premature or LBW infants, adolescentgirl, alcoholic and the elderly

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    PATHOLOGY

    Reduced O2 carrying capacity of the blood from nutrients

    deficiencies that disrupts hematopoiesis or hemoglobin

    synthesis

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    Etiology:

    - Decreased dietary intake

    - Decreased iron absorption from GIT

    - Chronic intestinal or uterine blood loss

    - Increased bodily needs for iron such as during growth period

    and pregnancy

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    SIGNS AND SYMPTOMS:

    Pallor Brittle hair, nails

    Easy fatigability Syncope

    Weakness Amenorrhea

    Anorexia Cold sensitivity

    Weight loss Tachycardia

    Headache/dizziness Palpitation

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    Planning

    - The patient will relate disease process and treatment

    - The patient will have increased circulation

    - The patient will relate measures to avoid injury

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    Nursing Intervention Explain the cause of disease, symptoms, side effect of medication

    and possible complication, and any procedures to be done

    Administer O2 as ordered check VS frequently

    Assess Homan signs and thrombi formation

    Positioning patient slowly to prevent dizziness

    Allow ADL if patient is able

    Provide HTP : to drink at least 3 L of H2O daily; elevate feet when resting;

    avoid restrictive clothing; wear support hose; take medication as ordered;

    report for chest pain, joint pain, fever or activity intolerance etc.

    Keep appointment for laboratory test and physician checks

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    Medical/surgical management

    Phlebotomy removal of blood from the vein about 350-

    500ml q other day til hct is 40%

    Radioactive phosphorus and radiation therapy to decreasethe production of blood cells in the bone marrow

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    Pharmacology

    Busulfan (Myleran); Chlorambucil (Leukeran);

    Cylophosphamide (Cytoxan) and mechlorethamine or

    nitrogen mustard = to decrease bone marrow production Allopurinol (Zyloprim) = to decrease production of uric acid

    Antihistamine = for pruritus

    Analgesic

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    Diet therapy

    Increased calories and protein

    Low in sodium to decrease fluid volume

    Avoid iron containing food

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    ANEMIAS

    Decreased availability of oxygen to the tissue

    TYPES of Anemia:

    1. Iron deficiency2. Folate deficiency

    3. Aplastic

    4. Pernicious

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    ETIOLOGY

    Acute/chronic blood loss

    Inadequate dietary intake of vitamins/minerals needed forRBC production

    Decreased RBC production by the bone marrow

    Increased destruction of RBC

    Increased demands of vitamins/minerals needed for RBCproduction

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    Signs and symptoms:

    Pallor easy fatigability Tachycardia

    weakness Palpitation

    anorexia Dizziness weight loss Headache

    syncope Paresthesia

    brittle hair and nails Amenorrhea

    Cold sensitivity

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    Iron Deficiency AnemiaDescription: Microcytic, hypochromic anemia

    From menses for females

    GI bleeding for men

    Assessment:- Vinson-Plummers Syndrome: stomatitis,dysphagia, atrophic glossitis (smooth,sore tongue)

    - Cheilosis (cracks at the side of the lips)

    - Koilonychia (spoon-shaped/concave fingernails)

    - Pica (craving of non-edible substance

    - Tinnitus

    - Cardiovascular symptoms 7.5g/dl:increase PR, chest pain

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    Nursing intervention

    Promote rest to reduce O2 demands

    Render oral care and good skin care

    Diet: iron rich food

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    Medical Mgt.

    o Iron supplement: oral FeSO4; parenteral Imferon

    o Oral should be given pc to prevent GI irritation

    o Oral liquid iron to be administered with straw to prevent permanent staining of theteeth

    o Vit. C increase iron absorptiono Do not administer with milk, antacid these will inhibit absorption

    o Inferon: Z-track to prevent staining of the skin

    o Do not massage site injection to prevent leakage of medication to subcu. layer

    o Iron salt change color of stool to dark green or black

    o Oxygen therapy

    o Blood transfusion

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    Folate deficiency anemia/megaloblastic anemia- Produced RBCs are abnormally large due to Vit 12 or folic acid

    deficiency

    Etiology:

    Poor dietary intake Prolonged TPN

    Eat Uncooked fruits and vegetables Malignancy

    Alcoholism Chronic Hemodialysis

    Chronic malnutrition Malabsorption

    Pregnancy Anorexia nervosa

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    Assessment Cracked lips, smooth, sore tongue and mild dirrhea

    Same as pernicious anemia

    Bone marrow analysis reveals hyperplasia

    Poikilocytosis(abnormally-shaped RBC)

    Extreme paleness, especially in mucous membrane Confusion, paresthesia in extremeties, lose of maintaining balance

    Lose of position sense (proprioception

    Mild jaundice, vilitigo, pemature of graying skin

    (+)Schilling test

    MANAGEMENT: Well balance diet, assess ambulation, gait and stability

    Folic acid 1mg/day; instruct to avoid extreme hot or cold

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    Pernicious anemia/B12 deficienccy Lack of intrinsic factor in gastric secretion

    Is an autoimmune disease in which parietal cells are

    destroyed and the gastric mucosa atrophies

    Macrocytic, hyperchromic anemia

    Onset 50 to 60 yrs. old

    ETIOLOGY:

    Gastric surgery

    Crohns disease

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    Pathophysiology

    Decreased Intrinsic factor production by the parietal cells of

    the stomach ->decreased Vit, B12 absorption ->decreased

    RBC production -> decrease DNA synthesis in maturing

    RBC -> impairment of cell integrity

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    Diagnostic test

    Schillings test

    most definitive diagnostic test

    - Oral radioactive Vit 12 is administered

    - IM nonradioactive Vit 12. To push the radioactive Vit 12 into

    the urine

    - Collect 24 hours urine specimen

    - Decreased excretion of Vit 12 in the urine indicates (+)

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    Assessment

    Beefy red, inflamed tongue ( most characteristic manifestation)

    Achlorhydria( absence of free Hcl acid in the gastric juice

    Tingling, numbness

    Lack of balance, uncoordinated movement

    Confusion

    Paralysis

    Depression, psychosis

    Jaundice

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    Collaborative MGT

    Vit 12 im: daily for 7 days; weekly for 10 weeks; monthly

    lifetime 100mg/Mos.

    Hydrochloric acid p.o. for 1 week

    Iron therapy

    Blood transfusion as needed

    Physical examination every 6 months

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    Aplastic anemia- Hypoplasia of the bone marrow

    - Fat replaces bone marrow

    - Pancytopenia drop in the # of RBC: anemia,

    leukopenia,thrombocytopenia

    ETIOLOGY: cause unknown

    - Congenital Medications Infection

    - Acquired heavy metals Hepatitis

    - Idiopathic

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    Diagnostic test

    Bone marrow aspiration/biopsy

    Adult: posterior iliac crest the most common site

    Prone position during the procedure

    Brief, sharp pain is normally experienced as bone marrow isaspirated

    Child: Tibia most common site

    Side-lying position during the procedure

    Apply pressure at the puncture site to prevent bleeding

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    Medical treatment

    -ATG (Atgam) antihymocyte globulin an Immunosuppressive

    therapy to suppress the rxn causing the aplastic anemia and

    allow the bone marrow to recover- Given OD through CVC for 7 to 10 days

    - Transfusion of plt. and Packed RBC

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    Surgical tx

    BM transplant

    Cyclosporine (sandimmune) an immnunosuppressant is

    given for BMT to decrease the graft rejection

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    Pharmacological tx

    Antibiotic for infection

    Steroids and Androgen used to stimulate the bone marrow

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    Acquired Hemolytic Anemia

    - Hemolysis or destruction of RBCS

    ETIOLOGY:

    Autoimmune rxn. arsenic

    Radiation lead

    Bld transfusion medication

    Chemicals clostridium perfringens

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    Signs and Symptoms

    Mild fatigue Pallor

    Elevated LDH Low Hb, Hct

    Pallor Jaundice

    Palpitation Hypotension

    Dyspnea Back and joint pain

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    MEDICAL MGT.

    BT- blood transfusion

    Erythrocytapheresis a procedure that removes abnormal

    RBCSand replaces a healthy one

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    Surgical tx

    Splenectomy to stop destruction of RBC

    Pharmacological:

    - Corticosteroids to decrease the autoimmune response

    - Folic acid to increase the production of RBC

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    SICKLE CELL Disease/INHERITED HYMOLYTICANEMIA

    Genetic disorder caused by recessive genes

    Has an abnormal Hb. S instead of Hb A in the RBC

    The person with one S gene (HbsA) has sickle cell

    trait

    The person with two S genes (Hbss) has sickle cell

    disease

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    Diagnostic Procedure

    Sickledex or sickle cell test

    - A screening test to infant to diagnose sickle cell disease

    - If Hb S is present, a Hb electrophoresis is done to distinguishbetween sickle trait and sickle cell disease

    - If the Hb electrophoresis test is negative, the client has thesickle cell trait and not sickle cell disease.

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    Factors can precipitate sickle cell crisis

    Dehydration Alcohol consumption

    Fatigue Blood Loss

    Infection Anesthesia Emotional stress Exposure to cold

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    Effect of sickle cell to circulatory system

    Obstruction of vessels and rupturing it and these become infarct andischemic that can cause: S &S

    Chronic anemia Dyspnea

    Cardiomegaly Arrhythmias

    Fatigue Fever

    Jaundice Severe pain

    Chronic leg ulcer Loss of bld supply

    Tachypnea Joint pain, swollen and tender

    priapism

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    Medical management

    Antibiotics Genetic Counselling

    Large amount of oral and IV fluid

    Pharmalogical:

    - Folic acid OD to assist in the production of RBC- Cetiedel citrate antisickling effect by changing the RBC membrane.

    - Trental (Pentoxifylline) reduces blood viscosity, increase RBCflexibility, lengthens the time between sickle cell crises

    - Blood transfusion during crisis

    - Patient-Controlled Analgesia with morphine during crisis

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    Nursing management Ineffective Tissue Perfusion r/t decreased # of RBC and decreased oxygenation

    as evidenced by dyspnea, tingling sensation and numbness of LE

    AdministerIV as ordered

    Offer 8 to 10 glasses of H20/day

    Monitor for symptoms of obstructed vessels such as: pain, legulceration, abdominal tenderness, dyspnea, confusion andblurred vision

    Administered Blood. product as ordered

    Close monitoring for possible BT RXN

    Elevate head; administer O2 as ordered

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    Alteration in comfort:Pain r/t occlusion of small vessels by sickled cellsas evidenced by---

    Assess the severity, location and type of pain

    Monitor analgesic administration by PCA pump

    Support joints and LE with pillows

    Keep bed linens off knees and ankles with a bed cradle

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    Activity Intolerance r/t imbalance between O2 supply and demand asE/B weakness, fatigue, tingling and numbness

    Assist with ADL

    Teach the importance of alternating periods of rest with

    activity

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    Knowledge deficit r/t prescribed TX, disease process

    Teach about the disease process

    Assist to take medication

    Explain the importance of avoiding stress and infection

    Stress the importance od adequate rest in a routine basis

    Explain to avoid high altitudes and nonpressurized airplane

    Discuss the important of avoiding tight-fitting -, restrictive clothing andstrenuous exercise, smoking and cold temp

    Advice to receive flue vaccine and pneumococcal vaccine

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    White blood disorder

    1. Leukemia

    - Is a malignancy of blood-forming tissues in which the bone

    marrow produces increased numbers of immature WBCthat are incapable of protecting the body from infections

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    Two classification of Leukemia1. Acute leukemia 2. Chronic leukemia

    subtype: subtype:

    a.)AML Acute myelocytic leukemia a.)ALLAcute lymphocytic leukemia

    -Malignant, affect monocytes, granulocytes, - Malignant proliferation of lymphoblasts

    erythrocytes and platelets arising from a disorder of a single

    common in adolescence and 55yrs of age lymphoid stem cell. Most common in

    children

    Survival: Survival:-1-3 yrs with chemo; 2-5 mos. if untreated - 5 yrs with chemo and radiation therapy;

    50% can be cured

    b.)CML Chronic myelocytic leukemia b.)CLL Chronic Lymphocytic Leukemia- caused byproliferation

    - Malignancy of the myeloid cell of small, abnormal B-lymphocytes

    that leads to uncontrolled proliferation WBC: between 20,000 and 100,000

    of granulocytes; common in adult develop with advanced age

    -Characterized by Philadelphia chromsomes

    -W

    BC count: 15,000-500,000

    Survival:3 -5 yrs if without BM transplant Survival: 5 7 yrs.

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    Pathophysiology

    - Proliferation ofImmatureWBCS----Immune system

    compromised state--production of RBCs and

    Platelets Hypertrophy of the Bone marrow (bonepain) organ Infiltration: hepatomegaly;

    Splenomegaly;renal insufficiency; hyperuricemia;

    arthralgia; Increase ICP (meningeal infiltration)

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    COLLABORATIVE MANAGEMENTMEDICAL MGT:

    Chemotherapy Bone marrow transplantation Blood transfusion

    NURSING INTERVENTION:

    PROTECT FROM INFECTION: Reverse isolation/protective isolation; Practice

    asepsis; Limit visitors; Do not allow people with infection to visit; Avoid fresh

    fruits/fresh flowers in the unit; Avoid raw foods

    PREVENTTRAUMA and BLEEDING: minimize parenteral injection, use small

    needle, apply pressure at injection site for 5 mins.,support/handle body parts

    gently,use soft-bristled toothbrush/soft swab, use electric razors, do not

    administer ASA

    CONSERVE ENERGY/O2 SUPPLY: adequate rest, Oxygen therapy

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    RELATIONSHIP OF LAB. TESTTO LEUKEMIA SYMPTOMS

    LAB RESULTS -- OVERALL SYMPTOMS -- SYMPTOMS MANIFESTATION

    Immature WBCs - Persistent infections -- fever, chills

    Decreased RBCs -Anemia

    --fatigue, pallor, malaise,tachycardia, tachypnea

    Decreased platelets --- Bleeding -- petechiae,bruising,

    melena, gingival bleeding,

    menorrhagia, epistaxis

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    Chemotherapeutic agents to treat leukemiaTYPES OF LEUKEMIA: CHEMOTHERAPEUTIC AGENTS:ALL Vincristine(oncovin)

    Prenisone(deltasone)

    6-mercaptopurine or 6-MP(purinethol)

    Methotrexate

    AML daunorubicin Hcl(cerubidine)

    cytarabine or era-C(cytosar

    -U)6-thioquanine(thioguanine)

    Doxorubicin Hcl(Adriamycin)

    CLL Chlorambucil(Leukeran),COP(cytoxan,oncovin,prednisone

    CML BUSULFAN(MYLERAN), HYDROXYUREA(HYDREA)

    DAT(daunorubicin,ara-C, thioguanine

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    AGRANULOCYTOSISDESCRIPTION - severely reduced number of granulocytes:basophils,

    eosinophils, neutrophils

    Etiology: medication toxicity, neoplastic disease, chemotherapy,

    radiation therapy, bacterial and viral infection

    Signs & symptoms:

    headache Chills Fever fatigue

    mucous membrane ulceration of the nose, mouth, pharynx, vagina,

    and rectum

    WBC and neutrophils is low

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    Collaborative Mgt.

    Culture and sensitivity if fever occur and ulceration

    Blood transfusion to provide mature leukocytes

    Filgrastin (Neopogen)- a human granulocyte colony

    stimulating factors

    Protective isolation

    Antibiotic specific for cultured microorganism

    Soft bland diet high in calories, protein and vitamins

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    Nursing intervention Infection control:

    Follow aseptic technique in any procedures

    Environment must be kept very clean

    Should avoid crowdsAvoid hot and cold temperature

    Client reports any signs or symptoms of infection

    Adequate amount of fluids

    Monitor wbc

    Provide rest between activities

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    COAGULATION DISORDERS:

    1. DISSEMINATED INTRAVASCULAR

    COAGULATION

    2. HEMOPHILIA

    3. THROMBOCYTOPENIA

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    DIC(DISSEMINATED INTRAVASCULAR COAGULATION)

    - A syndrome and not a disease

    - Occurs due to primary disease process or condition like:

    burns, acute leukemia, metastatic cancer, polycythemia vera,pheochromocytoma, shock, acute infection, septic abortion,

    abruptio placenta, BT rxn. and trauma

    - A condition of alternating clotting and hemorrhaging

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    Pathophysiology

    - Primary disease stimulation of clotting

    mechanism -- microthrombi formation process

    and Fibrinolysis

    Depletion of clotting factorsand Bleeding Obstruction of circulation

    tendency leads to organ and tissue necrosis. In

    bleeding - hemorrhage.

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    Signs and Symptoms Purpura( reddish purple patches on the skin) chest and

    abdomen

    Oozing of blood from venipuncture, mucous membrane,

    surgical wound Decreased urine output

    Increased prothombine time and Partial thromboplastin time,thrombin time

    Decreased fibrinogen and platelet count

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    Collaborative Mgt. Lab. Test D DIMER test measures a fibrin split product that is

    released when a clot breaks

    Treatment of primary disease. Ex. Infection antibiotic,CA chemotherapy

    Whole blood or blood products to normalize clotting factor levels

    Platelets and Packed RBC Cryoprecipitate or fresh-frozen plasma to nomalize clotting factor

    levels

    Heparin to prevent the formation of more microthrombi

    Aminocaproic acid (Amicar) to stop bleeding it inhibit the fibrinolyticprocess

    **Fibrinolysis is the process of breaking fibrin apart

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    Nursing Intervention Assess previous condition such infection, trauma, CA,

    MonitorI&O closely and record

    Monitor color, VS, peripheral pulses, neurological checks

    If abdominal bleeding is suspected measure abdominal girthevery 4 hours

    Assess surgical wounds, all body orifices for bleeding

    Assess presence of pulmonary edema, hypotension

    tachycardia, confusion restlessness, convulsion and coma

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    Hemophilia- Is an inherited bleeding disorder in which there is a lack of

    clotting factors

    - The trait is carried on the recessive X chromsome a

    mother is asymptomatic but can pass the trait to the sonwho manifest the symptoms

    TYPES:

    1. HEMOPHILIA A lacking of clotting factorsVIII

    2. HEMOPHILIA B (CHRISTMAS DISEASE) lacking of

    factorIX

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    Three Classification of Hemophilia

    1. Severe factor level less than 1% of

    normal

    2. Moderate factor level 1% to 5% normal

    3. Mild factor level 40% normal

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    Signs & Symptoms

    Bleeding main symptom

    Hemarthrosis bleeding into the joint -> causing pain

    swelling, redness and fever Spontaneous ecchymoses and bleed from the mouth and

    GIT & Urinary

    Complication: INTRACRANIAL HEMORRHAGE

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    COLLABORATIVE MGT. LAB. Test Partial thromboplastin time(PTT) result is prolonged

    Diagnosed by deficient or absent blood level of factors VIII orIX

    TX:

    administration of clotting factors VIII & IX

    Hemophilia A fresh frozen plasma and cryoprecipitate thathas factor VIII

    Hemophilia B - fresh frozen plasma that contain factorIX

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    Pharmacological

    Desmopressin acetate (DDAVP)

    Aminocaproic Acid (Amicar)

    ** To decrease fibrinolytic process

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    Nursing InterventionDeficient knowledge r/t disease process:

    Discuss ways to improve the safety of the clients home

    environment

    Advise not to take ASA

    Encourage to use electric razor and soft-bristled toothbrush

    Refer to genetic counselling

    Encourage client to wear Medic-Alert bacelet

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    Alteration in Comfort Pain r/t inflammation/swelling of tissue and joints

    Assess for bruising, swelling and joint discomfort

    Apply ice and pressure to bleeding site

    Immobilize with a supportive device

    Administer analgesic as ordered

    Risk forInjury r/t altered clotting factors: Transfuse clotting factors as ordered

    Encourage to avoid activities that may cause harm

    Post emergency medical # for future need.

    Prevent injuries: wear gloves, long-sleeved clothing when doinghousehold chores, participate in noncontact sport and activities

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    Thrombocytopenia

    - Is a decreased in the number of platelet in the blood

    - The decrease may R/T: aplastic anemia tumors, leukemia and chemotherapy

    Infection or viral illnessesIncreased platelet destruction as in DIC or thrombocytopenic purpura that

    is either drug induced or idiopathic( occurring without cause)

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    Signs and Symptoms Petechia Ecchymosis Bleeding from mucous membrane

    Very low Platelet count ; Prolonged Bleeding Time

    Hb, Hct is low; BMA shows mostly immature platelets

    Collaborative Mgt:

    Transfusion of platelet concentration Apheresis( removal of unwanted components) to remove the autoantibodies

    Splenectomy - primary site of platelet destruction

    Corticosteroids to prolong platelet life and strengthen the capillaries

    Immunosuppressive drugs, gamma globulin, and Vit K

    High fiver diet

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    Nursing intervention Activity should be undertaken to prevent trauma

    Assess level of pain on pain scale and coping ability

    Monitor VS, neuro andmental status

    Assess skin and excretions for signs of bleeding Handle very carefully in all nursing procedures

    Teach to use razor for shaving, soft bristles toothbrush,

    wear slippers at all time. Do not take ASA. Eat high fiver

    diet, blow nose very gently

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    Test questions Identification: Identify the ff: write the correct answer.1. Sickledex or Hb.Electrophoresis - are the diagnostic test for sickle cell anemia

    2. Anemia decreased number of RBCs and low Hb and hct level

    3. DIC is not a disease but a syndrome that occurs because of a clients having a primarydisease or condition

    4. Hemophilia is a recessive X chromosome inherited bleeding disorder in which the client islacking clotting factors

    5. Severe hemophilia one of the classification of hemophilia wherein the level factor is lessthan1% of normal

    6. Agranulocytosis a severely reduced number of granulocytes(basophil, eosinophils, andneutrophils

    7. Leukemia a malignancy of blood forming tissues in which the bone marrow producesincreased numbers of immatureWBC

    8. Phlebotomy one of the treatment of polycytemia which is the removal of blood from a vein

    9. Polycythemia is a disease in which there is an increased production of RBC

    10. Iron deficiency Anemia - the most common type of anemia and occurs when the body doesnot have enough iron to synthesize functional Hb.

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    Enumeration