Hematologic Malignancies. Anna Schaal, RN, MSN, arnp Norris Cotton Cancer Center Lebanon, New Hampshire

Transcription

1 Hematologic Malignancies Anna Schaal, RN, MSN, arnp Norris Cotton Cancer Center Lebanon, New Hampshire

2 Objectives At the end of the session, the oncology nurse will be able to: Explain the pathophysiology of hematologic malignancies Discuss the rationale for treating the client with a hematologic malignancy List common nursing interventions for patients with hematologic malignancies

3 Hematopoiesis

4 Hematologic Malignancy Risk Factors Cause is unknown Elevated risk associated with Immunodeficiency organ transplants, autoimmune disorders Infectious agents HIV/AIDS: adult T cell lymphoma EBV: Burkitt s lymphoma, PTLD HCV: Marginal zone lymphoma Helicobacter pylori: MALT lymphomas Environmental exposures Drugs, chemicals, radiation, occupational exposures Genetic Predisposition Downs syndrome

5 Leukemia Epidemiology Approximately 52,380 cases of leukemia will be diagnosed in 2014 (ACS, 2014) Leukemia occurs in more adults than children (ACS, 2014) More common in males ALL: most common form of childhood leukemia (ACS, 2014)

6 Pathophysiology of Leukemia Malignant disorder of blood cells and lymphatic tissues Leukemic, malignant cells excessively proliferate Leukemic cells capable of infiltrating and accumulating in other organs

7 Pathophysiology Symptoms of leukemia attributed to: Type of leukemia cell Degree of leukemic cell burden Degree of myelosuppression Effects of organ involvement

8 Types of Leukemia Acute leukemia: adults and children Acute Myelogenous Leukemia (AML) Acute Lymphocytic Leukemia (ALL) Chronic leukemia Chronic Myelogenous Leukemia (CML) Chronic Lymphocytic Leukemia (CLL) Hairy Cell Leukemia (HCL)

9 Types of Leukemia Has to do with cell lineage and maturity

10 V Faderl S, Talpaz M, Estrov Z, O Brien S, Kurzrock R, Kantarjian HM. N Engl J Med. 1999;341: Faderl S, Talpaz M, Estrov Z, Kantarjian HM. Ann Intern Med. 1999;131:

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13 AML De novo vs. secondary (alkylating agents) Risk related to age and cellular biology Immunophenotyping, cytogenetics Initial remission rates 60 70% (adults) Median survival with treatment months

14 ALL Most common childhood leukemia Peak age 2 5 yrs 80% cured 80% are B cell, 20% T cell In adults is highly aggressive Long treatment programs 2 3 years CNS infiltration common

15 Leukemia: Clinical Presentation Usually associated with symptoms of cytopenias

16 Leukemia Management Depends on the type and stage AML Induction: Initial treatment with chemotherapy agents given at high doses to eradicate leukemia Consolidation,, which may include hematopoietic stem cell transplantation

17 Leukemia Management ALL Induction Initial treatment with chemotherapy agents given at high doses to eradicate leukemia Post remission therapy Consolidation therapy Intensification therapy Maintenance therapy +/ Stem cell transplant

18 Leukemia Management CLL Watch and wait Chemotherapy and biotherapy May include allogeneic stem cell transplant CML Tyrosine Kinase Inhibitors (oral) May include allogeneic stem cell transplant

19 Leukemia Management: Stem Cell Transplant Autologous Patients own stem cells Is a rescue for high dose chemotherapy Allogeneic Donor cells Depends on graft vs. leukemia affect Myeloablative vs. non myeloablative

20 Non Hodgkin s Lymphomas: Overview Heterogeneous neoplasms Differing patterns of growth, treatment response Multiple sites, extranodal lesions common Reed-Sternberg cell, Wright-stained

21 Lymphatic System Lymphatic vessels, nodes and organs Primary Lymphoid organs Bone marrow Thymus Secondary Lymphoid organs Lymph nodes Spleen Tonsils, adenoids (Waldeyer s ring) Digestive and respiratory tracts (MALT)

22 Pathophysiology: Lymphocytes T cells Release cytokines B cells Produce antibodies Natural killer (NK) cells Kill infected cells Attack cancer cells Non Hodgkin lymphoma 85% B cells 15% T cells

23 Incidence per 100, NHL: Age Demographics Age

24 Aggressiveness of Mature B Cell NHL Type Lymphomas Treatment Goals Indolent or slow-growing Aggressive CLL/SLL WM MZL FL MCL DLBCL PMBL Treatable, but not curable Goal is to control and minimize symptoms Waxing and waning course Transformation to large cell lymphoma can occur Without treatment, life expectancy is short Potential long-term survival with treatment Most treated with curative intent Cure rates vary CLL=chronic lymphocytic leukemia; DLBCL=diffuse large B-cell lymphoma; FL=follicular lymphoma; MCL=mantle cell lymphoma; B-lymphoblastic MZL=marginal zone lymphoma; PMBL=primary mediastinal large B-cell lymphoma; Treated SLL=small with lymphocytic curative leukemia; intent WM=Waldenstrom s macroglobulinemia. Very aggressive Precursor lymphoma/leukemia Burkitt s lymphoma/ B cell acute leukemia Cure rates vary

25 NHL: Common Subtypes BL (< 1%) Other subtypes (9%) MCL (6%) FL (22%) T and NK cell (12%) DLBCL (31%) SLL/CLL(7%) MALT-type MZL (7.5%) Nodal-type MZL (< 2%) Lymphoplasmacytic (1%) MCL = mantle cell lymphoma; FL = follicular lymphoma; SLL = small lymphocytic lymphoma; CLL = chronic lymphocytic leukemia; MALT = mucosa-associated lymphoid tissue; MZL = marginal zone lymphoma; BL = Burkitt lymphoma.

26 Ann Arbor Staging System Stage I Single lymph node group Stage II Multiple lymph nodes on same side of diaphragm Stage III Multiple lymph nodes on both sides of the diaphragm Stage IV Multiple extranodal sites or lymph nodes and extranodal disease Substaging: Extranodal extension (E) Systemic symptoms (A or B) Bulk > 10 cm (X)

27 International Prognostic Index Adverse Risk Factors Age >60 Stage 3 or 4 >2 extranodal sites Performance Status >2 LDH>normal Risk Category Low = 1 risk factor Low intermediate 2 High intermediate 3 High 4 or 5

28 Hodgkin s Disease Clonal lymphoid malignancy Characterized by Reed Sternberg cells

29 Epidemiology of Hodgkin s Disease Incidence: In 2018, approximately 8500 estimated new cases in USA Expect 1050 deaths HD comprises 16% of all lymphomas, but less than 1% of all new cancers Age: Incidence is bimodal (greatest peak in age range with a second smaller peak after age 60 years) Sex: Male to female ratio is 1.4 to 1

30 Prognostic factors Hemoglobin < 10.5g/dL Male Age < 45 years Stage IV disease Leukocytosis (WBC count > 15,000/mm 3 ) Lymphocytopenia (ALS < 600/mm 3 ) Freedom from disease progression at 5 years: 0 features = 84% (7%) 1 features = 77% (22%) 2 features = 67% (29%) 3 features = 60% (23% ) 4 features = 51% (12%) 5 or more = 42% (7%)

31 Lymphoma: Clinical Presentation Painless lymphadenopathy B symptoms Fever, night sweats, weight loss Constitutional symptoms [fatigue] Bone marrow involvement Site specific findings A Swollen Lymph Node in the Neck 31

32 Treating Lymphoma

33 Multiple Myeloma Malignant proliferation of plasma cells in the bone marrow accompanied by an increased production of abnormal immunoglobulin (IgG, IgA, IgD, IgM) Destroys bone tissue Decreases bone marrow function Affects the immune system

34 Hematopoiesis

35 Pathophysiology: Plasma Cells Terminally differentiated cells of B lymphocyte lineage Cellular factories whose job is devoted to producing a single antibody protein Normally incapable of dividing Abundant in lymph nodes and bone marrow

36 Epidemiology Gender: Males > Females (1.3:1 ratio). Age: Onset of disease is late, with peak incidence at age > 60 years, fewer than 3% age <40 Race: Incidence is greater in African Americans than in Caucasians (2:1 ratio). Geography: No clear geographic distribution although higher relative incidence in more developed countries.

37 International Staging System (ISS) Better Response to Therapy Stage I Factors: beta-2 microglobulin <3.5 mg/dl Albumin 3.5 g/dl Most Favorable Prognosis Stage II Factors: beta-2 microglobulin <3.5 mg/dl Albumin <3.5 g/dl or beta-2 microglobulin <5.5 mg/dl Lesser Response to Therapy Stage III Factors: beta-2 microglobulin 5.5 mg/dl Less Favorable Prognosis

38 CRAB Symptoms of MM C Calcium R Renal dysfunction A Anemia B Bone pain or lesions

39 Multiple Myeloma Clinical Presentation Back Pain Anemia Hypercalcemia Renal Failure Infections Fatigue

40 Treating Multiple Myeloma Approached as a chronic disease Treatable, but not curable

41 Diagnostic Evaluation: History and Physical Clinical evaluation Document onset of suspicious symptoms, acute episodes of illness, adenopathy, historical labs Review of medication profile Co-morbid conditions Physical exam Clinical significance May indicate onset of disease, rapidity of symptom onset and progression, presence of B-symptoms (fever, night sweats, weight loss, pruritus) Identification of any drug-induced cytopenias or symptoms, complexity of co-morbid conditions, potential drug interactions Effective management of co-morbid conditions may play a critical role in selecting therapies Underlying cardiovascular disease may require dose modification or omission of anthracyclines Establish a baseline for adenopathy, organomegaly, other extramedullary sites of disease, and identification of any abnormal findings which may require immediate intervention

42 Peripheral Blood Diagnostic study Clinical significance CBC + differential + platelets Reticulocyte count LDH, haptoglobin, coombs, and reticulocyte count LDH Serum β 2 m (lymphoma and MM) Hepatic profile Evaluate presence of cytopenias, lymphocytosis, morphological abnormalities, and bone marrow response to anemia Evaluate for underlying hemolysis Necessary for risk stratification using IPI Evaluate for aggressive disease, risk for TLS, and hemolysis Prognostic relevance Reflects WBC membrane turnover Levels are affected by renal function Treatments have potential renal and hepatic toxicities or may be affected by renal or hepatic insufficiencies Serum albumin reflects nutritional status and used to estimate prognosis

43 Diagnostic Evaluation: Peripheral Blood Diagnostic study Clinical significance Quantitative immunoglobulins UA, LDH, K+, PO 4, Ca++ Serum iron, ferritin, TIBC, folic acid, B12 Hepatitis B screen Lumbar puncture with cerebrospinal fluid analysis Hypogammaglobulinemia is common in aggressive disease and associated with an increased risk of infections Baseline tumor lysis screen Evaluate for other possible causes of anemia Risk of Hepatitis B reactivation a) HBsAg positive, particularly those who are HBeAg positive or have high levels of HBV DNA male gender b) Use of corticosteroids; use of rituximab Should be considered in patients with high LDH, multiple extranodal sites, epidural masses, testicular disease, paranasal or nasopharyngeal disease

44 Diagnostic Evaluation: Tissue Biopsy * Definitive diagnosis for lymphoma can only be made biopsy of pathologic LN or tumor tissues Diagnostic study Morphology Immunohistochemistry Flow cytometry Molecular profiling Clinical significance Review of cytology using low-power microscope to define basic architecture of the lymphatic tissue Excisional biopsy is the standard for initial diagnosis of NHL (fine needle aspirates are felt to be inadequate) Used to isolate cellular proteins which correlate with phases of B-cell differentiation Immunophenotyping used to describe antigen expression on B-cells using peripheral blood and bone marrow Used to correlate with the tissue biopsy for WHO classification of subtype Newer molecular profiling has identified key prognostic markers as well as potential targets for new therapies

45 Diagnostic Evaluation: Bone Marrow Diagnostic study Aspirate Should include spicules and be cellular enough to assess at least 500 cells Biopsy Should be of adequate size for evaluation (1 2 cm) Cytogenetics Clinical significance Evaluation of morphological abnormalities of hematopoietic precursors to allow WHO classification Used for flow cytometry, FISH analysis, and cytogenetics Evaluate cellularity, topography, presence of lymphocytic infiltrates, exclusion of other bone marrow disorders or bone marrow infiltration by solid tumors Evaluate for possible non-random chromosomal abnormalities Usually based on evaluation of 20 metaphases Greater than 2 metaphases is considered non-random

46 Diagnostic Evaluation: Imaging Diagnostic study MUGA scan or echocardiogram CT chest, abdomen, and pelvis Clinical significance _ Baseline evaluation for patients receiving anthracycline therapy Current standard of care for initial staging on NHL Estimation of anatomic extent of disease and areas of abnormal lymph nodes (> 1 cm) 18 FDG-PET PET with FDG shows functional metabolic status Useful in evaluation of lymph nodes < 1 cm Chest X-ray Skeletal Survey Baseline evaluation for any underlying disease Evaluate for lytic lesions, plasmacytomas, impending fractures

47 Supportive Therapy Nursing Interventions Infection Early recognition and prevention of infection IV immunoglobulin therapy for recurrent infections Consider yearly influenza vaccine Consider PCP, herpes, and antifungal prophylaxis with use of highdose Dexamethasone regimens Use of myeloid growth factors - neupogen Anemia Early recognition and treatment Erythropoietin therapy Red blood cell transfusions Thrombocytopenia Prevention of bleeding Platelet transfusions

48 Supportive Therapy Nursing Interventions Calculating the ANC WBC 0.8 Neutrophils 27 Immatures (bands) 1.2 (PMN s + Bands) X WBC

49 Supportive Therapy Nursing Interventions Constipation/Diarrhea Assessment of bowel habits changes in fluid and dietary intake and activity medication administration Pain Assessment and documentation Proper positioning and supports Consultation with PT/OT Analgesics Non-pharmacologic therapies Cord compression Proper positioning and supports Consultation with PT/OT Vertebroplasty

50 Supportive Therapy Nursing Interventions Nausea and Vomiting Anti-emetics remember anticipatory and delayed Monitor renal function/hydration status Complimentary non-pharmacologics ie.. Guided imagery, music therapy, accupressure Renal dysfunction (may occur in 25% MM patients) Maintain hydration to avoid renal failure Allopurinol Avoid NSAIDs and IV contrast dye Monitor for renal dysfunction with chronic use of bisphosphonates Not a contraindication to transplant Plasmapheresis may slow down/prevent renal failure by removal of M- protein from the blood

51 Supportive Therapy Nursing Interventions Cognitive impairment Counseling regarding injury prevention; anxiolytics, antidepressants Hypercalcemia (25% MM patients at presentation) Avoid/discontinue calcium supplements Hydration plus diuretics Bisphosphonates, steroids and//or calcitonin Bone disease (most common MM presenting symptom) Regular monitoring of bone lesions with skeletal survey or focused CT scan/mri Bisphosphonate administration intravenously q 3-4 weeks Regular exercise Avoid situations that put one at risk to fall Analgesia

52 Ann Arbor Staging is used in what type of malignancy? A. Lung Cancer B. Colon Cancer C. Breast Cancer D. Lymphoma

53 Mr. Jones has multiple myeloma and is currently in an unmaintained remission. Which of the following s/sx would indicate that his disease may be progressing? A. a drop in his total protein B. frequent urination C. pain in his right forearm D. an elevated BUN

54 Miss Jones has a total WBC of 2.0 with 48% neutrophils and 2% bands. What is her ANC? A. 550 B C. 100 D. 5000

55 Miss Jones has newly diagnosed NHL. She has a postive pet scan with adenopathy in the cervical, axillary and inguinal areas. Her bone marrow biopsy was negative for disease. She has no other extranodal disease sites. What stage of NHL does she have? A. 1 B. 2 C. 3 D. 4

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