Nrsg 200 leukemia

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Chapter 33Management of Clients

with Leukemia and Lymphoma

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Cancers of the Hematopoietic System

• Result from proliferation of malignant cells originating in:– Bone marrow– Thymus– Lymphatic tissue

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Hematopoietic Cell Disorders: Leukemia: Etiology & Risk Factors– Leukemia: malignant disease of blood-

forming organs (bone marrow)– Common feature: unregulated proliferation

or accumulation of WBCs in bone marrow, replacing normal marrow elements

– Proliferation in liver, spleen, & lymph nodes & invasion of nonhematologic organs

– Cause is unknown; some evidence of genetic influence, viral pathogenesis, or radiation/chemical exposure•Alkylating agents

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Hematopoietic Cell Disorders: Leukemia: PathophysiologyClassified according to:• Cell line involved

– Lymphoid– Myeloid

• Maturity of malignant cells– Acute (undifferentiated cells)

•Onset of sx is abrupt; progresses rapidly– Chronic (mature cells)

•Sx evolve over months to years

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Hematopoietic Cell Disorders: Leukemia

• Acute leukemia: – acute lymphocytic leukemia (ALL) – acute myeloid leukemia (AML)

• Chronic leukemia: – chronic myeloid leukemia (CML) – chronic lymphocytic leukemia (CLL)

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Hematopoietic Cell Disorders: Acute LeukemiaAcute myeloid

leukemia (AML)• Defect in

hematopoietic stem cell

• Affects all ages; increases with age

• S/S: insufficient normal blood cells:– Infection

(neutropenia)– Weakness/fatigue

(anemia)– Bleeding

(thromobocytopenia)

• Prognosis:– Older pts have

worse prognosis– Secondary form

R/T alkylating agents have poorer prognosis

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AML: Complications• Major causes of death:

– Infection•Risk increases with severity of

neutropenia– Risk of fungal infection increases

– Bleeding•Major bleeding can occur with platelets

less than 10,000•Common sites are GI, pulmonary,

intracranial

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Treatment of AML• Goal is remission• Induction Therapy: Aggressive chemo

– Requires several weeks hospitalization– Aim is to eradicate leukemic cells but also

eradicates normal cells– Patient severely neutropenic, anemic &

thrombocytopenic---very ill!– Mucositis causes diarrhea, inability to eat– Cytosar, Ara-C, Cerubidine, Novantrone

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Tumor Lysis Syndrome • Due to massive leukemic cell

destruction & release of intracellular electrolytes & fluids into systemic circ.

• Increased uric acid & phosphorus– Increased risk of renal stones/ ARF

• Increased potassium/ low Ca++– Cardiac dysrhythmias– Muscle cramps, weakness, tetany,

confusion, seizures

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Tumor Lysis Syndrome --- Tx

• Increased fluids• Alkalinization of urine (sodium

bicarb)• Allopurinol to prevent

crystallization of uric acid

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Tx of AML---cont’d.• Supportive care

– Blood products (PRBCs & platelets)

– Treat infections• Neupogen or Leukine can

shorten period of neutropenia

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Alternative Treatments• BMT (Bone Marrow Transplant)• PBSCT (Peripheral Blood Stem

Cell transplant)– Aggressive chemo (& radiation) to

destroy patient’s bone marrow followed by infusion of donor stem cells

– At risk for graft-versus-host reaction

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Post-remission Therapy• Consolidation therapy

– To eliminate any residual leukemia cells

– Multiple treatment cycles, often with same agents used for induction but at lower doses

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Chronic Myelogenous Leukemia (CML)

• Malignancy of myeloid stem cells---increased blast cells

• Bone marrow expands into cavities of long bones & cells formed in liver & spleen– Enlarged, tender spleen & liver

• Milder than acute with more normal cells present

• 90-95% genetic abnormality—”Philadelphia chromosome” 22 translocated onto chromosome 9

• Uncommon under 20 yrs of age

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CML: Clinical Manifestations

• Sx variable; some are asymptomatic• Very high leukocyte counts may lead

to SOB, mild confusion• Enlarged, tender spleen• Malaise, anorexia, weight loss• 3 stages:

– Chronic– Transformation– Blast crisis: Sx worsen as disease progresses

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Treatment of CML—chronic stage

• Oral administration of Gleevec (tyrosine kinase inhibitor)

• Oral chemo agents Hydrea, Myleran• Leukapheresis: Leukocytes removed

from patient’s blood, then returned • Interferon-alfa, cytosine

– Given SQ daily– Fatigue, depression, anorexia,

mucositis, inability to concentrate

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CML----Transformation Stage

• This stage marks the progress toward acute form (blast crisis)

• May have bone pain, fevers• Enlarged spleen• Worsening anemia,

thrombocyopenia

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CML---blast crisis• Tx similar to induction therapy

for acute leukemias– Lymphoid blast crisis more likely

to re-enter chronic phase• Life-threatening infections &

bleeding occur frequently

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Treatment-- CML• Potential cure with bone

marrow transplant or PBSCT– Greater chance of cure if done

during chronic phase– Considered for pts younger than

65• Imatinib (Gleevec) therapy can

decrease need for transplant

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Acute Lymphocytic Leukemia (ALL)

• Uncontrolled malignant proliferation of lymphoblasts (esp. B-cell precursor)

• 20% of blast cells have chromosome translocation similar to CML

• Most common in young children– Males > females– Peak at 4 yrs– Uncommon after age 15

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ALL• Immature lymphocytes crowd

development of normal cells; inhibiting hematopoiesis

• Leukocyte counts may be high or low but always high % of immature cells

• Infiltrate liver, spleen, CNS (leading to H/A & vomiting)

• Bone pain• 80% survive 5 yrs

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Treatment of ALL• Goal is complete remission• Corticosteroids• Vinca alkaloids• May include cranial irradiation or

intrathecal MTX for CNS involvement• Imatinib used for those with chromosome

abnormality• Monoclonal antibodies being studied• BMT or PBSCT may be done if illness

recurs

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Chronic Lymphocytic Leukemia (CLL)

• 2/3 are older than 60• Most common type of leukemia

in US & Europe—rare in Asia• Occurs more often in males

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CLL--pathophysiology• Most cells are mature but escape apoptosis,

resulting in excessive accumulation of cells in bone marrow & circulation

• Elevated lymphocyte count in early stages, more than 100,000

• Lymphocytes trapped in lymph nodes: Enlarged painful nodes with enlarged liver & spleen

• Anemia & thrombocytopenia occur in later stages

• Autoimmune complications: hemolytic anemia or ITP (idiopathic thrombocytopenic purpura)

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CLL---S&S• Many are asymptomatic• Increased lymphocyte count• Lymphadenopathy• Splenomegaly, hepatomegaly• “B” symptoms

– Fever, night sweats, weight loss– Defects in humoral & cellular immunity

•Anergy with skin testing• Infections are common

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CLL--Treatment• No tx required in early stages• Later stages or symptomatic:

– Monoclonal antibodies being used more (Rituxan, Campath)•At risk for infection: Septra + antivirals

used x 2 months after tx ends– Fludara, steroids or Leukeran

•S/E Leukeran: bone marrow suppression with risk of opportunistic infections

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Diagnostic Findings• Bone marrow

aspiration—increase marrow & immature

• Lumbar puncture—blast cells in CSF

• Chest x-ray, MRI, CT—to detect lesions & sites of infection

• CBC count varies greatly; WBC may be very hi or very low– Anemia– Thrombocytopenia– Leukopenia

• Lymphangiography, Lymph node biopsy—locate malignant lesions & classify

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Acute & Chronic Leukemia: Nursing Diagnoses• Ineffective Protection; Risk for Infection r2

neutropenia, chemotherapy, radiation therapy

• Ineffective Protection; Risk for Hemorrhage r2 thrombocytopenia 22 treatment

• Imbalanced Nutrition: Less Than Body Requirements r2 lack of appetite, nausea & vomiting; gi proliferative changes, chemo

• Deficient Fluid Volume r2 decrease in intake; cancer process; chemotherapy

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Acute & Chronic Leukemia: Nursing Diagnoses--cont’d• Fatigue & Activity Intolerance r2 anemia• Disturbed Body Image; Sexual

Dysfunction r2 change in appearance, in function, and roles

• Ineffective Therapeutic Regimen Management r2 length, complexity, expense of Tx

• Impaired Skin Integrity r2 toxic effects of chemotherapy

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Nursing Considerations• Mucositis: oral + rectal areas

– Oral assessment & hygiene with soft toothbrushes or swabs

– Rinses with NS or baking soda– Tx of yeast in mouth (Mycelex,

Diflucan)– Cleanse & dry peri area

thoroughly

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Nursing Considerations• Nutritional Intake

– Mouth care before & after meals– Analgesics before eating– Anti-emetics– Small frequent meals of soft foods at

moderate temperature– Avoid uncooked fruits & vegs– Nutritional supplements– Daily weights/ I&O– May require parenteral nutrition

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Nursing Considerations• Pain & comfort

– Tylenol to reduce fever– Sponging with cool water for fever

(avoid cold water or ice packs)– Back & shoulder massage to ease

muscle aches– PCA– Interrupted sleep

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Nursing Considerations• Fatigue

– Balance activities & rest– Sit in chair rather than lying in bed– HEPA filter mask may allow

neutropenic patient to walk hallways

– Consider PT/ stationary bikes– Encourage self-care

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Nursing Considerations• Anxiety

– Emotional support/ providing information

– Depression/ grief common– Support spiritual well-being

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Nursing Considerations: Bleeding Precautions

• Assess skin, GI, GU, neuro status, VS, heart & lungs every shift– Test urine, emesis & stools for occult blood

• Monitor CBC/ platelets• Avoid ASA• No IM injections, rectal temps or supp.• Use stool softeners• Hold venipuncture sites x 5 minutes• No flossing of teeth/ use soft toothbrush• Avoid suctioning• Electric razor• Avoid vigorous nose blowing• Pad side rails, prevent falls

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Nursing Considerations: Neutropenic Precautions

• Assess signs of infection every shift: skin, mouth, lungs, GI, GU, neuro, VS including temp

• Monitor CBC. Call MD for neutrophil count less than 1000

• Monitor CXR, C&S reports

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• No one with URI or sore throat should visit or care for patient

• Care for neutropenic pt. first if possible• Needs private room if ANC less than

1000• No fresh flowers• Room cleaned daily• Change water in containers every 24

hours (e.g. O2 humidification bottles)

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• Eliminate fresh fruits & veg. or salads• No suppositories, rectal temps or

enema• Deep breathing every 4 hours while

awake• Ambulate: HEPA mask in halls• Prevent skin dryness• Meticulous hygiene including peri care

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• Oral hygiene after meals & every 4 hrs

• Do not use plastic IV catheters if ANC is less than 500; central line preferred

• Inspect IV site every shift; use aseptic technique for dressing changes

• Give antimicrobials on time

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Lymphomas

– General term for group of cancers originating in lymphatic system

– Results when lymphocyte (WBC) undergoes malignant change & multiplies, crowding out healthy cells & creating tumors which enlarge lymph nodes or other sites

– Tumors of primary lymphoid tissue (thymus & bone marrow)

– Secondary lymphoid tissue (lymph nodes, spleen, tonsils, & intestinal lymphoid tissue)

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Lymphomas -- 2 categories

• Hodgkin’s Disease—specialized form; initiates in a single node & spreads along lymph system– Viral etiology suspected (EBV)

• Non-Hodgkin’s (NHL’s)—heterogeneous groups of cancers that originate from neoplastic growth of lymphoid tissue– Most involve B-lymphocytes– Spread is unpredictable– Now 6th most common type of cancer in US– Incidence increased in immunodeficiencies, prior CA tx

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S&S of Hodgkin’s Lymphoma

– Painless enlargement of one or more lymph nodes on one side of the neck

– Mediastinal mass on CXR– Pruritus (cause unknown)– All organs are vulnerable to invasion

• Cough, jaundice, abdominal or bone pain• “B” symptoms: fever, sweats, weight loss

– Mild anemia, platelets usually WNL– Leukocyte count high or low– Elevated sed rate– Impaired cellular immunity (skin tests)– Herpes zoster infections are common

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Hodgkin’s Lymphoma

• Diagnostic Findings– Lymph node biopsy: distinct, large

cell—Reed-Sternberg cells– Bone marrow biopsy– Chest x-ray– CT/ PET scan– Bone scan

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Treatment of Hodgkin’s• Goal is cure• Tx based on stage of disease• 2-4 months of chemo followed by

radiation (early stages may receive radiation alone)– Adriamycin, bleomycin, vinblastine &

dacarbazine (“ABVD” therapy)• Recurrent disease treated with high-

dose chemo then BMT

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Survival Rates• Hodgkin’s lymphoma one of most

curable forms of cancer• Many cured after 1 treatment• 5-yr survival 86% all races• 5-yr survival 95.2% for 0-14 yr• Can develop other cancers (lung,

breast)---encourage healthy lifestyle to avoid risks

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NHL—Clinical Manifestations

– Generalized or localized lymphadenopathy; most common in cervical, axillary, inguinal, femoral

– Painless– May wax & wane

in less aggressive types

– S/S diffuse NHL variable & more systemic

– Night sweats, fever, wt loss (“B” symptoms)

– Hepato-or-splenomegaly

– Lymphomatous masses can compromise organ function

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NHL: Medical Management

• Tx based on classification & stage of disease

• Early stage, localized NHL sometimes treated with radiation

• Widespread disease requires chemotherapy or combined treatment

• Tx after relapse is controversial: BMT or PBSCT may be done if less than 60 years old

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Survival Rate• 5-year survival rate increased

from 31% in whites (1960-1963) to 63.8% for all races

• 5-year survival rate for children, 0-19, 83.5%

• Est. 19,730 deaths from lymphoma in 2007 (18,660 NHL/1070 HL)

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Multiple Myeloma• Malignant disease of plasma cells

(most mature form of B-lymphocyte) which produce increased amount of non-functional immunoglobulin (M-protein)

• Total protein levels are elevated• Abnormal plasma cells also secrete

substance to stimulate angiogenesis

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Multiple Myeloma---S&S• Bone pain in back or ribs which increases

during the day• Lytic lesions seen on x-ray; may develop

spinal fractures• Hypercalcemia: thirst, constipation, confusion• Renal failure due to proteins damaging renal

tubules• Anemia: Fatigue, weakness• Serum hyperviscosity with bleeding from nose

or mouth, or heart failure

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Multiple Myeloma---Diagnosis

• Bence-Jones protein on serum protein electrophoresis, or in urine

• Lytic bone lesions on x-ray• Bone marrow biopsy confirms

dx: presence of sheets of plasma cells

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Multiple Myeloma----Tx• No cure, even with BMT or PBSCT• Chemo is primary treatment

– Steroids, Alkeran, Cytoxan, Adriamycin• Radiation may strengthen bone at a

specific lesion if at risk for fx or spinal compression

• Bisphosphonates, vertebroplasty for compression fx

• Plasmapheresis for hyperviscosity• Thalidomide inhibits angiogenesis

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Nursing care for Multiple Myeloma

• Pain control: NSAIDs or opioids, body mechanics, braces

• Teach about S&S hypercalcemia– Hydration important

• Monitor renal function; dialysis may be necessary

• Bacterial infections common due to impaired antibody production– Needs flu & Pneumovax vaccines– Watch & report signs of infection

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Bone Marrow Transplantation• Indications—aplastic anemia,

malignant disorders, non-malignant hematologic disorders, immunodeficiency disorders

• Bone marrow types– Allogeneic (donor other than patient)– Syngeneic (from identical twin)– Autologous (from patient)

• Histocompatability testing for allogeneic & syngeneic transplants

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Bone Marrow Transplantation: Types

Allogeneic• from relative or unrelated donor

with close HLA match • Previously most common • BUT highest rate m/m due to

complications of incompatibility• Increased use of autologous &

peripheral stem cell transplants

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Syngeneic• Donated by identical twin• Perfect HLA match• Eliminates rejection risk• Higher rate of leukemic

relapse (GVHD has anti-leukemic effect)

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Autologous• Used for pts without donors or those

who require ablative doses of chemo for an aggressive malignancy

• Eliminates risk of immunologic response

• Relapse is frequent– Contamination of bm by malignant cells?– Failure of pre-transplant chemo?

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Allogeneic Transplant

• Donor preparation—extensive work-up– Collection: informed consent

• Marrow collection• Peripheral stem cell collection

• Recipient preparation (“conditioning regimen”)– Malignant cells destroyed– Marrow cavities emptied– Bone marrow infused

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• Graft-versus-host disease: most common & potentially disastrous complication of allogeneic BMT & may occur 7 to 30 days after infusion– Acute: staged R/T organ system affected

•Skin, liver, GI, pulmonary, encephalopathy– Chronic: occurs ~ 100 days post-

transplant; resembles autoimmune collagen-vascular disorders (SLE; scleroderma; Sjogren’s); severe case is often fatal

GVHD

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GVHD• Donor’s immune cells (T cells)

attack body of “host”• Reduce by

– Close match of donor– Drugs to prevent (cyclosporine &

methotrexate)– Removing T cells from donated cells

• Early Tx = better outcomes

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Post-BMT care• 1st 100 days after BMT are crucial: immune

system & hematopoiesis recovering• High risk of death from infection or

bleeding• Also at risk for venous occlusive disease

after high-dose chemo• At risk for tumor lysis syndrome & acute

tubular necrosis• Total body irradiation often leads to

sterility, cataracts

Nrsg 200 leukemia

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