This document provides an overview of cancers of the hematopoietic system, including leukemia and lymphoma. It discusses the etiology, pathophysiology, signs and symptoms, diagnostic findings, treatment approaches, nursing considerations, and outcomes for various types of leukemia (acute myeloid leukemia, chronic myelogenous leukemia, acute lymphocytic leukemia, chronic lymphocytic leukemia) and lymphomas (Hodgkin's lymphoma, non-Hodgkin's lymphoma, multiple myeloma). It also covers bone marrow transplantation as a treatment option.

1. Chapter 33 Management of Clients with Leukemia and Lymphoma

2. Cancers of the Hematopoietic System Result from proliferation of malignant cells originating in: Bone marrow Thymus Lymphatic tissue

3. 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

4. Hematopoietic Cell Disorders: Leukemia: Pathophysiology Classified 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

5. Hematopoietic Cell Disorders: Leukemia Acute leukemia: acute lymphocytic leukemia (ALL) acute myeloid leukemia (AML) Chronic leukemia: chronic myeloid leukemia (CML) chronic lymphocytic leukemia (CLL)

6. Hematopoietic Cell Disorders: Acute Leukemia Acute 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

8. 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

9. 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

10. 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

11. Tumor Lysis Syndrome --- Tx Increased fluids Alkalinization of urine (sodium bicarb) Allopurinol to prevent crystallization of uric acid

12. Tx of AML---cont’d. Supportive care Blood products (PRBCs & platelets) Treat infections Neupogen or Leukine can shorten period of neutropenia

13. 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

14. 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

15. 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

16. 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

17. 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

18. CML----Transformation Stage This stage marks the progress toward acute form (blast crisis) May have bone pain, fevers Enlarged spleen Worsening anemia, thrombocyopenia

19. 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

20. 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

21. 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

22. 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

23. 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

24. 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

25. 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)

26. 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

27. 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

28. 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

29. Acute & Chronic Leukemia: Nursing Diagnoses Ineffective Protection; Risk for Infection r 2 neutropenia, chemotherapy, radiation therapy Ineffective Protection; Risk for Hemorrhage r 2 thrombocytopenia 2 2 treatment Imbalanced Nutrition: Less Than Body Requirements r 2 lack of appetite, nausea & vomiting; gi proliferative changes, chemo Deficient Fluid Volume r 2 decrease in intake; cancer process; chemotherapy

30. Acute & Chronic Leukemia: Nursing Diagnoses--cont’d Fatigue & Activity Intolerance r 2 anemia Disturbed Body Image; Sexual Dysfunction r 2 change in appearance, in function, and roles Ineffective Therapeutic Regimen Management r 2 length, complexity, expense of Tx Impaired Skin Integrity r 2 toxic effects of chemotherapy

31. 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

32. 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

33. 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

34. 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

35. Nursing Considerations Anxiety Emotional support/ providing information Depression/ grief common Support spiritual well-being

36. 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

37. 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

38. Nursing Considerations: Neutropenic Precautions 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)

39. Nursing Considerations: Neutropenic Precautions 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

40. Nursing Considerations: Neutropenic Precautions 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

41. 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)

42. 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 6 th most common type of cancer in US Incidence increased in immunodeficiencies, prior CA tx

43. 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

44. 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

45. 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

46. 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

47. 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

48. 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

49. 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)

50. 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

51. 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

52. 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

53. 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

54. 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

55. 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

56. 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

57. Bone Marrow Transplantation: Types Syngeneic Donated by identical twin Perfect HLA match Eliminates rejection risk Higher rate of leukemic relapse (GVHD has anti-leukemic effect)

58. Bone Marrow Transplantation: Types 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?

59. 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

60. 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

61. 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

62. Post-BMT care 1 st 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