NSG210 Hinkle Ch. 30: Management of Patients With Hematologic Neoplasms

Source: Hinkle & Cheever, Brunner & Suddarth’s Medical-Surgical Nursing 15e, Chapter 30, pp. 950-982 (textbooks/HINKLE_MS_CH30.pdf, also in textbooks/exam03/). Week 09 reading (cancer, lung cancer and chest tubes, non-Hodgkin lymphoma); on Exam 3. Numbering note: the syllabus lists “Ch. 31,” but in the 15th edition Ch. 31 is immune function assessment; lymphoma (Hodgkin and non-Hodgkin), leukemia, and multiple myeloma are in Ch. 30.

Learning Outcomes

1. Compare the leukemias by incidence, physiology, manifestations, complications, and management.
2. Use the nursing process for the patient with acute leukemia.
3. Explain the myeloproliferative disorders and their management.
4. Compare Hodgkin and non-Hodgkin lymphomas.
5. Discuss medical and nursing care of the patient with multiple myeloma.

I. Overview

• Hematopoiesis starts with the hematopoietic stem cell (HSC) in the marrow; it splits into myeloid (RBCs, platelets, granulocytes, monocytes) and lymphoid (B and T lymphocytes) lines
• Leukemia: uncontrolled proliferation of WBCs (often immature) that crowds the marrow
• Lymphoma: neoplasm of lymphoid tissue, usually B lymphocytes
• Multiple myeloma: malignancy of the plasma cell, the most mature B lymphocyte
• Key terms: blast cells (immature leukocytes), pancytopenia (low WBC, RBC, platelets), neutropenia, thrombocytopenia, petechiae, splenomegaly, lymphadenopathy, indolent (slow-growing)
• Absolute neutrophil count (ANC) = total WBC x percent neutrophils (segs plus bands)

II. Leukemia

• Classified by cell line (myeloid vs lymphoid) and by speed (acute vs chronic)
• Acute: abrupt onset over weeks; development halted at the blast phase; death in weeks to months without treatment
• Chronic: evolves over months to years; mostly mature cells
• Risk factors: radiation, chemicals (benzene), prior chemotherapy, genetic disorders (Down syndrome), viruses
• Leukocytosis that persists or keeps rising is abnormal and needs workup

A. Acute myeloid leukemia (AML)

• Most common leukemia and most common cause of leukemia death; median age about 68; more common in males
• Risk: age, benzene/pesticides, radiation, prior alkylating chemo, smoking, Down syndrome
• Manifestations come from marrow failure:
  • Neutropenia: fever, infection
  • Anemia: pallor, fatigue, weakness, dyspnea on exertion, dizziness
  • Thrombocytopenia: bruising, petechiae, epistaxis, gingival bleeding
  • Infiltration: gum hyperplasia, bone pain, enlarged liver/spleen; lymphadenopathy rare
• Diagnosis: CBC (low RBC, platelets; WBC low, normal, or high) and bone marrow with more than 20% blasts (hallmark)
• Acute promyelocytic leukemia (APL, AML-M3): high risk of fatal coagulopathy (DIC) but highly curable
• Treatment:
  • Induction: high-dose cytarabine plus daunorubicin, idarubicin, or mitoxantrone; hospitalized 4-6 weeks; ANC may reach zero; platelets under 5,000
  • Consolidation: after counts recover, to kill residual cells (usually cytarabine)
  • Allogeneic HSCT (HLA-matched donor) after conditioning chemo/radiation
  • Older or frail: lower-intensity therapy (hypomethylating agents, low-dose cytarabine, hydroxyurea) or supportive care only (death in months from infection or bleeding)
  • G-CSF (filgrastim) or GM-CSF (sargramostim) only for life-threatening infection; not in APL (differentiation syndrome risk)
• Complications:
  • Bleeding (major hemorrhage risk when platelets under 10,000): GI, pulmonary, vaginal, intracranial; DIC especially in APL
  • Infection (neutrophils under 100 dramatically raise systemic infection risk); bacterial and fungal; antifungal prophylaxis
  • WBC over 100,000 causes leukostasis in brain and lungs
  • Tumor lysis syndrome (TLS): high uric acid, potassium, phosphate, low calcium; kidney stones, AKI, arrhythmias, tetany, seizures; prevent with high fluid intake and allopurinol or rasburicase

B. Chronic myeloid leukemia (CML)

• Philadelphia chromosome: BCR-ABL fusion gene from a 9;22 translocation causing excess tyrosine kinase
• 15% of leukemias; average age 67
• Three phases: chronic (few symptoms, found on routine CBC), accelerated (worsening counts, splenomegaly, bone pain, fever), blast crisis (behaves like acute leukemia)
• WBC over 100,000 may cause dyspnea or confusion (leukostasis); enlarged tender spleen
• Treatment: tyrosine kinase inhibitors (TKIs) - imatinib is standard; dasatinib, nilotinib first-line alternatives; bosutinib, ponatinib second-line
  • Imatinib: periorbital edema; dasatinib: myelosuppression, pleural effusion, prolonged QT; nilotinib: cardiotoxic
  • All metabolized by CYP450: grapefruit juice, clarithromycin, azole antifungals raise levels; corticosteroids, antiseizure drugs, antacids, St. John’s wort lower levels
• Allogeneic HSCT can cure younger patients (90% disease free 5 years if done in chronic phase)
• Nursing: oral TKI adherence is only about 51%; teach schedule, alarms, side effects, safe handling; monitor ECG (QT), urine output, CBC

C. Acute lymphocytic leukemia (ALL)

• 75-80% of cases in children; peak age 4; boys more than girls; rises again after 45
• 75% B-cell, 25% T-cell; BCR-ABL in 20%
• Infiltration more common than other leukemias: liver/spleen enlargement, bone pain; CNS involvement (cranial nerve palsies, headache, vomiting); testes, breasts
• Treatment: induction, consolidation, maintenance plus intrathecal chemo for CNS prophylaxis
  • Corticosteroids (dexamethasone preferred, better CNS penetration) and vinca alkaloids; anthracycline; asparaginase (thrombosis risk)
  • TKIs for Philadelphia-positive ALL; blinatumomab, inotuzumab for relapse
  • CAR-T cell therapy: patient’s own T cells modified and reinfused
• Steroid side effects: infection (viral), avascular necrosis

D. Chronic lymphocytic leukemia (CLL)

• Most common adult leukemia in the Western world; average age 71
• Strong familial link (10% with a relative); Agent Orange exposure in Vietnam veterans
• Mature malignant B cells that escape apoptosis; accumulate in marrow, blood, nodes, spleen
• Often found incidentally; lymphocytosis always present; lymphadenopathy common, splenomegaly
• B symptoms signal worse prognosis; elevated beta-2 microglobulin means advanced disease
• Autoimmune hemolytic anemia or ITP; Richter’s transformation to aggressive lymphoma in 2-10%
• Infections cause over half of deaths: herpes zoster, encapsulated organisms (H. influenzae); low immunoglobulins - IVIG for recurrent infection
• Treatment: watch and wait if asymptomatic; anti-CD20 antibodies (rituximab) plus chemo (fludarabine most common - prolonged marrow suppression, PCP, CMV, herpes risk); ibrutinib for TP53 deletion; alemtuzumab (anti-CD52) needs antiviral and TMP-SMX prophylaxis for months
• Teach: pneumonia and flu vaccines, NO live vaccines, annual skin exam (higher skin cancer rate)

E. B symptoms (Chart 30-1)

• Fever 100.4 F (38 C) or higher not explained by infection
• Drenching night sweats
• Unintentional weight loss of 10% or more over 6 months
• Seen in CLL, Hodgkin, and NHL; indicate worse prognosis

III. Nursing Process: The Patient With Acute Leukemia

• Assess daily: subtle signs of infection (dry cough, mild dyspnea, diminished breath sounds) - infection may not show on chest x-ray because there are no neutrophils to create an inflammatory response
• Monitor WBC, ANC, Hct, platelets, creatinine, electrolytes, coagulation, liver tests; report culture results immediately
• Diagnoses: risk for infection and bleeding, impaired oral mucosa, impaired nutrition, pain, fatigue, fluid imbalance, self-care deficit, anxiety and grief, spiritual distress
• Complications: infection, bleeding/DIC, renal dysfunction, TLS, cardiac toxicity, infertility
• Interventions:
  • Neutropenic and bleeding precautions (Ch. 12, Chart 12-6)
  • Mucositis: mouth care before and after meals, analgesics before eating; warn about biting tongue with oral anesthetics
  • Nutrition: small, frequent, soft, moderate-temperature foods; low-microbial diet (weak evidence); daily weights, calorie counts, parenteral nutrition if needed
  • Fever: acetaminophen, tepid sponging - avoid cold water or ice (vasoconstriction traps heat)
  • Fatigue: balance activity and rest; HEPA mask to walk outside room; sit up in chair
  • Fluids: strict I&O, daily weight; replace potassium and magnesium (amphotericin depletes electrolytes)
  • Self-care: nurse performs hygiene when patient cannot, but encourage independence
  • Anxiety and grief: assess how much information the patient wants, reassess over time; realistic hope; offer pastoral care
  • Discharge: vascular access device care, home care, when to seek care; death usually from infection, less often bleeding; discuss hospice options

IV. Myelodysplastic Syndromes (MDS)

• Clonal myeloid stem cell disorders with dysplasia and cytopenias; may progress to AML
• Older adults (median 65-70); hypercellular marrow but cells die before release; cells function poorly (infection and bleeding even with near-normal counts)
• Diagnosis: macrocytic anemia; bone marrow aspiration and biopsy; IPSS risk score
• Treatment: transfusions, erythropoiesis-stimulating agents, hypomethylating agents (azacitidine, decitabine), lenalidomide for deletion 5q; allogeneic HSCT is the only cure
• Iron overload from repeated transfusions: keep ferritin under 1,000; oral chelator deferasirox (take in evening before dinner; diarrhea, cramping; watch creatinine; not with liver disease)
• Pneumonia is most common infection; prophylactic antibiotics not routine
• Teach infection and bleeding precautions; tell dentists and other providers about MDS

V. Myeloproliferative Neoplasms (Philadelphia-negative)

A. Polycythemia vera

• Hypercellular marrow; RBCs predominate (Hct can exceed 60%), also high WBC and platelets; JAK2 mutation; median age 60
• Hyperviscosity: headache, dizziness, vision changes, TIAs; ruddy complexion, angina, claudication, hypertension; early satiety (splenomegaly)
• Pruritus (especially after water contact), erythromelalgia (burning red fingers/toes), elevated uric acid (gout, stones)
• Thrombosis (stroke, MI) is the most common cause of death; bleeding also possible
• Treatment: phlebotomy (mainstay) to keep Hct under 45% (500 mL once or twice weekly initially); low-dose aspirin for all; hydroxyurea for high risk (age 60 or older or prior thrombosis); interferon-alfa (under 60 or pregnant); ruxolitinib (JAK2 inhibitor)
• Nursing: tepid baths, pat dry, oatmeal or cocoa butter lotions; reduce CV risk factors; avoid crossing legs and tight clothing; AVOID iron supplements (including multivitamins); limit alcohol; hold aspirin if bleeding

B. Essential thrombocythemia

• Platelets persistently over 450,000 (can exceed 1-2 million); women twice as often; median age 65-70
• Symptoms from vascular occlusion (headache most common neurologic symptom, TIAs, erythromelalgia) and bleeding (when platelets over 1.5 million - acquired von Willebrand deficiency)
• Rule out iron deficiency (reactive thrombocytosis)
• Treatment by risk: low-dose aspirin; hydroxyurea, interferon, anagrelide for high risk; platelet apheresis for acute crisis

C. Primary myelofibrosis

• Rarest; marrow fibrosis, extramedullary hematopoiesis; massive splenomegaly in about 90% (early satiety, cachexia)
• Allogeneic HSCT is the only therapy that reverses fibrosis; ruxolitinib; splenectomy can cause reactive thrombocytosis
• Nursing: small, frequent high-calorie, high-protein meals; energy conservation (Table 30-3); fatigue in up to 94%

VI. Lymphoma

A. Hodgkin lymphoma

• Rare, high cure rate (about 90%); peaks at 15-34 and after 60; slightly more males
• Risks: Epstein-Barr virus, HIV, family history, immunosuppression, Agent Orange
• Unicentric: starts in a single node and spreads contiguously along lymph chains
• Reed-Sternberg cell (giant, “owl-eye” cell from B lymphocytes) is the diagnostic hallmark
• Subtypes: nodular sclerosis (most common, about 70%, young), mixed cellularity (older, HIV), lymphocyte-depleted, lymphocyte-rich, nodular lymphocyte-predominant (popcorn cells, not classical)
• Presentation: painless, firm, enlarged node on one side of the neck (cervical most common); mediastinal mass may compress trachea; B symptoms in about 40%; pruritus; pain in nodes after drinking alcohol; herpes zoster common
• Diagnosis: excisional lymph node biopsy; PET scan most sensitive for staging; CT chest/abdomen/pelvis; MUGA or ECG before anthracyclines
• Ann Arbor staging:
  • Stage I: single node region or single organ
  • Stage II: two or more node regions on the same side of the diaphragm
  • Stage III: node regions above and below the diaphragm
  • Stage IV: widespread, multiple organs
  • A = no symptoms; B = B symptoms; E = extralymphatic; S = spleen
• Treatment by stage (not histology): ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine) with or without radiation; relapse: brentuximab, nivolumab, pembrolizumab
• Late effects: second cancers (lung, breast; more than 10 years later, especially women with chest radiation), cardiovascular disease, hypothyroidism (about 50% after neck radiation), infertility, premature menopause, fatigue
• 5-year survival 92-94% localized, 78% distant

B. Non-Hodgkin lymphoma (NHL)

• Heterogeneous; 85% B-cell, 15% T-cell or NK; 7th most common US cancer; median age 66; more males
• Spread is unpredictable; true localized disease uncommon; extranodal sites common
• Risks: immune deficiency, autoimmune disease, organ transplant, prior cancer treatment, EBV, HIV, HHV8, herbicides/pesticides, Agent Orange
• Indolent (about 40%, e.g., follicular - 5-year survival about 90%) vs aggressive (e.g., diffuse large B-cell - 72%, Burkitt)
• Presentation: painless node swelling in neck, axilla, groin; about one third have B symptoms; most diagnosed late
  • Mediastinal mass: cough, dyspnea, chest pain; abdominal mass: bowel obstruction, ureter compression (AKI); splenomegaly: early satiety
• Diagnosis: incisional or excisional node biopsy, flow cytometry, FISH, CT, PET, bone marrow if needed; prognosis by IPI (FLIPI for follicular)
• Treatment goal is remission (cure is the goal in Hodgkin)
  • Indolent localized: radiation alone or watchful waiting
  • Aggressive: R-CHOP (rituximab plus cyclophosphamide, doxorubicin, vincristine, prednisone); CNS involvement needs intrathecal chemo or cranial radiation
  • Relapse: ICE, CAR-T (axicabtagene), autologous HSCT
• Complications:
  • Tumor lysis syndrome 12-72 hours after starting therapy, highest risk with bulky disease, Burkitt, DLBCL
  • Hepatitis B reactivation with rituximab - screen and give lamivudine or entecavir
  • Progressive multifocal leukoencephalopathy (confusion, weakness, vision and speech changes) - 90% fatal
• Nursing: know the type, stage, and treatment plan; mostly outpatient care; infection teaching; fatigue; survivorship (screen for second cancers like AML/MDS, cardiac and fertility follow-up, no smoking, healthy weight, 150 minutes exercise a week)

VII. Multiple Myeloma

• Malignant plasma cells make a nonfunctional monoclonal immunoglobulin (M protein); normal antibodies decrease
• About 17% of hematologic malignancies; median age about 70; African Americans twice the risk; may evolve from MGUS (0.5-1% per year)
• CRAB features:
  • hyperCalcemia (over 11.5 mg/dL): thirst, dehydration, constipation, polyuria, confusion, coma
  • Renal dysfunction (creatinine over 2 or CrCl under 40): Bence Jones proteins, hypercalcemia, amyloid, hyperviscosity; 33% at diagnosis, 50% eventually
  • Anemia (Hgb under 10): marrow crowding plus low erythropoietin
  • Bone destruction: osteoclast activating factor and IL-6; lytic lesions in long bones, vertebrae, skull; vertebral collapse, loss of height, kyphosis, spinal cord compression
• Classic symptom: bone pain in back or ribs that worsens with movement and improves with rest
• Safety alert: any older adult with back pain and elevated total protein should be evaluated for multiple myeloma
• Diagnosis: elevated total protein, Rouleaux formation, serum protein electrophoresis (M spike) or free light chain assay, beta-2 microglobulin and LDH (tumor burden), imaging for lytic lesions, bone marrow biopsy
• Treatment (no cure; control symptoms and prolong life):
  • Smoldering: observe every 3-6 months
  • Active: bortezomib (proteasome inhibitor) or daratumumab (monoclonal antibody) based regimens with lenalidomide/thalidomide and dexamethasone; then autologous HSCT (standard of care); lenalidomide maintenance
  • All patients get a bisphosphonate (pamidronate, zoledronic acid) - strengthens bone, prevents hypercalcemia; teach dental care to prevent osteonecrosis of the jaw
• Complications:
  • Infection (low normal immunoglobulins, not neutropenia): S. pneumoniae, H. influenzae; zoster and PCP with steroids - TMP-SMX and acyclovir prophylaxis; pneumococcal and flu vaccines
  • Hyperviscosity (oncologic emergency): nose/mouth bleeding, headache, blurred vision, paresthesias, HF - hydration and plasmapheresis
  • Spinal cord compression (most common neurologic complication)
  • Peripheral neuropathy up to 75% (bortezomib, thalidomide) - report early so dose can be cut; give bortezomib subcut rather than IV
  • VTE (about 10%), higher with immunomodulators plus steroids - aspirin or anticoagulant prophylaxis
• Nursing:
  • Pain: NSAIDs cautiously (renal and GI risk), long-acting opioids
  • Keep urine output about 3 L/day; monitor creatinine; mobility and hydration reduce hypercalcemia
  • Lift no more than 10 lb, proper body mechanics, braces as needed to prevent pathologic fracture
  • Neuropathy safety: avoid extreme water temperatures, inspect feet, remove scatter rugs, walker, slow position changes (orthostatic hypotension)
  • Older adults: goals may favor symptom control and independence over survival

High-Yield Numbers

• AML diagnosis: more than 20% blasts in marrow
• Major hemorrhage risk: platelets under 10,000; induction platelets often under 5,000
• Neutrophils under 100: dramatic rise in systemic infection
• WBC over 100,000: leukostasis (brain, lungs)
• Induction hospitalization: 4-6 weeks
• ALL: peak age 4; 75-80% children
• CLL average age 71; AML 68; CML 67
• CML oral TKI adherence about 51%
• B symptoms: fever 100.4 F, night sweats, 10% weight loss in 6 months
• Polycythemia vera: keep Hct under 45%; phlebotomy 500 mL
• Essential thrombocythemia: platelets over 450,000; bleeding when over 1.5 million
• MDS chelation: ferritin under 1,000
• Hodgkin cure about 90%; nodular sclerosis about 70%; B symptoms about 40%
• NHL: 85% B-cell; TLS 12-72 hours after chemo
• Myeloma CRAB: calcium over 11.5, creatinine over 2, Hgb under 10, lytic lesions; urine output 3 L/day; lift no more than 10 lb

Priority Nursing Actions and Red Flags

• Neutropenic patient with any fever: treat as infection, cultures and antibiotics promptly; signs of infection are blunted (no pus, clear chest x-ray)
• Bleeding precautions with low platelets; watch for GI, pulmonary, intracranial bleeding; DIC in APL
• Tumor lysis syndrome after starting chemo: hydrate, allopurinol or rasburicase, watch potassium, phosphate, calcium, uric acid, urine output, ECG
• Leukostasis (very high WBC): dyspnea, confusion
• Fever: tepid sponging, no ice packs
• No live vaccines in CLL and other immunosuppressed patients
• Polycythemia vera: no iron supplements; tepid water for pruritus
• Older adult with back pain plus high total protein: think multiple myeloma
• Myeloma: new or worse back pain or leg weakness may be spinal cord compression; confusion and constipation may be hypercalcemia; nose/mouth bleeding and blurred vision may be hyperviscosity
• Report neuropathy early; ECG and MUGA before anthracyclines; ECG for QT with TKIs
• Rituximab: screen for hepatitis B
• Immunomodulators (thalidomide, lenalidomide): pregnancy tests, strict contraception, VTE prophylaxis

Medications

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