INTERNAL MALIGNANCY: LEUKEMIA/LYMPHOMA
A. Definition: Cutaneous lesions may develop with internal hematopoietic and lymphoid malignancies, which are classified by dominant cell type, cell maturity, and duration from onset to death (acute vs. chronic). Leukemia classifications describe the type of white blood cell (WBC) multiplying while lymphomas develop from lymph node, spleen, or other organ lymphocytes. Some classifications include the following:
1. Acute myelomonocytic involves monocytosis in the peripheral blood.
2. Monocytic leukemia affects the reticuloendothelial (macrophage) system with an excess of phagocytic cells, macrophages, and monocytes in connective tissues that overrun the blood and accumulate in the lymph nodes and spleen.
3. Acute lymphocytic leukemias exhibit lymphoblast proliferation and enlarged lymphoid tissues, while aleukemic leukemias have no abnormal cells in the blood.
4. Acute myeloblastic leukemia has a proliferation of immature myeloblasts in tissues, organs, and blood; frequently, it is associated with Sweet’s syndrome (SS).
5. Chronic hairy cell leukemia shows hairy cells in reticuloendothelial organs/blood.
6. Non-Hodgkin lymphoma represents multiple cytologic classifications, other than Hodgkin disease, of either nodular or diffuse tumor pattern.
B. Etiology. Unknown for both leukemias and lymphomas; however, some studies may indicate a possible genetic, environmental, or viral link.
1. Leukemia is characterized by neoplasia of the WBC in the bone marrow, interfering with normal blood cell maturation and resulting in anemia, decreased platelet production, and granulocytopenia.
2. Non-Hodgkin lymphomas show an abnormal proliferation of various cell types—histiocytic, lymphocytic, or mixed. Malignant cells may spread to lymph nodes, bone marrow, liver, spleen, and gastrointestinal (GI) tract.
3. Tumor cells may directly invade the skin manifesting as papules, macules, flat or infiltrated plaques, nodules, or ulcerative skin lesions.
4. As an indirect result of the malignancy, skin lesions may manifest as pallor (anemia), excoriations (pruritus), petechiae (thrombocytopenia), and opportunistic infections such as severe candidiasis (with neutropenia).
1. Leukemia accounts for 3.1% of all new US cancers and has an annual prevalence reported at 9 per 100,000.
2. Non-Hodgkin’s Lymphoma (NHL) is a broad spectrum of lymphomas. It is three times more common than Hodgkin lymphoma. The majority of cutaneous lymphomas are of T-cell origin due to their function in the skin and lymphoid tissues.
E. Considerations across the life span
1. Acute leukemia is most common in children, while chronic leukemia is more common in adults in the third to sixth decade of life.
2. Non-Hodgkin lymphoma is most common in the fifth and sixth decades of life and it accounts for 7.0% of all childhood cancers.
A. History and physical examination
1. A careful review of systems is essential to determine constitutional symptoms such as headaches, fatigue, fever, malaise, easy bruising, hemorrhages, bone or joint pain, and nutritional deficits (anorexia and weight loss with lymphomas with added nausea, vomiting, and diarrhea in leukemias).
2. Skin examination from leukemias and lymphomas may demonstrate both specific and nonspecific lesions.
Common physical examination findings may be hepatomegaly, splenomegaly, painless lymphadenopathy (lymphomas or leukemias), and musculoskeletal- or neurological-related pain and weakness (headaches, visual changes, vertigo, tinnitus in leukemia and pain/paresthesias/paralysis of affected nerve pathways) (Figure 21-1
4. Clinical hematologic abnormalities include anemias, thrombocytopenia, local/systemic infections (respiratory and skin with leukemias), and opportunistic infections.
B. Specific skin changes
Most common are rubbery, erythematous/purplish papules, plaques, or nodules, randomly distributed and/or found in mucous membranes. Less common are fleshcolored lesions (Figures 21-2
Least common are lesions similar in appearance to mycosis fungoides (flat plaques, arciform papules, and ulcerated nodules or tumors, most pruritic in plaque stages) (Figures 21-7
3. Early skin changes are most common in acute myelomonocytic and monocytic leukemia and include infiltrated, hyperplastic, friable gingiva, bruising or petechiae, and slow healing of cuts.
4. Aleukemic leukemia cutis presents in the skin as erythematous papules or nodules in the absence of malignant cells in the peripheral blood; Leder stain is the key to diagnosis.
C. Nonspecific skin changes
1. Skin and mucous membrane findings attributable to cytopenia include pallor, purpura, gingival bleeding, oral ulcerations, and recurring skin infections.
2. Skin changes in which the pathogenesis is unknown include acute febrile neutrophilic dermatosis (Sweet’s syndrome), atypical pyoderma gangrenosum, generalized pruritus, vasculitis, acquired ichthyosis, vesicular disease, and xanthomas.
D. Differential diagnosis
1. Differentials include viral, bacterial, and fungal infections seen in tuberculosis, cat-scratch fever, sarcoidosis, dermatomyositis, lymphogranuloma venereum, mononucleosis-type syndromes, human immunodeficiency virus (HIV) infection, Kaposi sarcoma, secondary syphilis, serum sickness, and Kawasaki disease.
2. Pseudolymphomas of the skin may manifest as insect infestations or bites, lymphomatoid papulosis, druginduced pseudolymphoma, actinic reticulosis.
E. Diagnostic tests
1. General diagnostic tests include CBC with differential; HIV; rapid plasma reagin (RPR); hepatitis B surface antigen (HBsAg); antinuclear antigen (ANA); mononucleosis serology; purified protein derivative (PPD) skin test; metabolic panel with lipid profile; x-rays/scans of the chest, abdomen, and bone; and, when indicated, lymph node and/or bone marrow biopsy.
FIGURE 21-1. Massive lymphadenopathy in a patient with chronic lymphocytic leukemia. (From Tkachuk, D. C., & Hirschman, J. V. (2007). Wintrobe’s atlas of clinical hematology (p. 154, Fig. 5.1). Philadelphia, PA: Lippincott Williams & Wilkins.)
FIGURE 21-2. Lymphoma/leukemia—purple/red plaques and papules of leukemia cutis—extensor area of arms. (Image provided by Stedman’s.)
FIGURE 21-3. Leukemia cutis in an infant with congenital leukemia. (From Arceci, R., & Weinstein, H. (2005). Neoplasia in the neonate and young infant. In G. B. Avery, (Ed.). Neonatology: Pathophysiology and management of the newborn. Philadelphia, PA: JB Lippincott, with permission.)
FIGURE 21-4. Gingival infiltration of leukemic cells in a patient with acute myeloid leukemia. (From Greer, J. P., Foerster, J., Rodgers, G. M., Paraskevas, F., Glader, B., Arber, D. A., & Means Jr, R. T. (2009). Wintrobe’s clinical hematology (12th ed., p. 1680, Fig. 72.8). Philadelphia, PA: Lippincott Williams & Wilkins.)
FIGURE 21-5. Ocular involvement in a 60-year-old man with chronic lymphocytic leukemia. (From Shields, J. A., & Shields, C. L. (2015). Eyelid, conjunctival, and orbital tumors: An atlas and textbook. Philadelphia, PA: Wolters Kluwer.)
FIGURE 21-6. Cutaneous B-cell lymphoma. Asymptomatic erythematous lavender plaques and tumors on the face and scalp. (From Elder, D. E. (2012). Atlas and synopsis of lever’s histopathology of the skin. Philadelphia, PA: Wolters Kluwer.)
FIGURE 21-7. Cutaneous T-cell lymphoma. Note “smudgy patches.” (Reproduced with permission from Goodheart, H. G. (2009). Photoguide to common skin disorders: Diagnosis and management (3rd ed.). Philadelphia, PA: Lippincott Williams & Wilkins.)
FIGURE 21-8. Cutaneous lymphoma of hand. (From Dr. Barankin Dermatology Collection.)
FIGURE 21-9. Cutaneous T-cell lymphoma: mycosis fungoides with follicular papules and plaques on face. (From Lugo-Somolinos, A., Lee, I., McKinley-Grant, L., Goldsmith, L. A., Papier, A., Adigun, C. G., …, Fredeking, A. (2011). VisualDx: Essential dermatology in pigmented skin. Philadelphia, PA: Wolters Kluwer.)
FIGURE 21-10. Large non-Hodgkin rectal lymphoma eroding through the perianal skin. (From Corman, M. (2012). Corman’s colon and rectal surgery. Philadelphia, PA: Wolters Kluwer.)
2. Skin biopsy will demonstrate specific type of malignant cell but must be correlated with the history, physical exam, and other diagnostic data.
III. COMMON THERAPEUTIC MODALITIES
A. Systemic therapies include multiple modalities—chemotherapy, radiation, immunotherapy, and bone marrow transplant.
1. Supportive systemic therapies possible are antibiotic prophylaxis, blood/platelet transfusions, and/or use of bone marrow growth factors (erythropoietin, granulocyte-macrophage colony-stimulating factor [GM-CSF]).
B. Local therapies utilized are skin radiation therapy (SRT) to specific lesions or intralesional corticosteroid injections.
1. Supportive management includes topicals for pruritus, oral ulceration or bleeding, and emollients and moisturizers after showering for xerosis.
C. Medical interventions treat primary and secondary disease
1. Clinically assess and monitor supportive blood/platelet replacement transfusions when needed.
2. Monitor skin lesions, blood, urine, and pulmonary systems for potential sources of infection; when indicated, obtain appropriate wound, body fluid, or skin cultures for suspected infections.
D. Medication/drug therapy use and monitoring when indicated
1. Add supportive therapy with antibiotic prophylaxis or treatment of infection.
2. Bone marrow growth factors (erythropoietin or human GM-CSF) to treat anemias and prevent fatigue/malaise.
3. Apply topical anesthetic creams to manage possible oral ulcerations.
4. Administer nonaspirin antipyretic or apply cooling blanket for fever.
E. Diet management
1. Promote adequate fluid intake, multivitamin use, and well-balanced diet and add high-protein foods when skin breakdown is evident.
2. Encourage daily intake of vitamin B complex, to include B6, B12, and folate, supplementation due to high bone marrow turnover with leukemias.
3. Encourage frequent, small feedings of soft nonspicy, nonacidic foods when oral ulcerations are present.
4. Cooled foods may be better tolerated until ulcerations resolve.
F. Surgical intervention
1. Selective splenectomy may be utilized to manage chronic lymphocytic leukemia.
G. Supportive skin care
1. Maintain mobility and body positioning to promote skin integrity and prevent skin breakdown.
2. Use nondeodorant, mild skin cleansing agent.
3. Apply emollients to lips to prevent chapping, dryness, bleeding, or pain.
4. Lubricate skin with cream- or ointment-based skin care product after bathing.
5. If incontinent, keep skin clean and dry and use barrier ointment to prevent chaffing, breakdown, and candida organism overgrowth.
6. Assess and treat opportunistic skin infections like candidiasis or herpes infections.
H. Other nursing interventions
1. Maintain good pulmonary toilet to prevent respiratory illness.
2. Maintain healthy oral cavity; identify oral ulcers or lesions for topical treatments.
3. Prevent constipation or bladder infections with effective bowel/bladder elimination plans.
4. If neutropenic or during acute exacerbation, consider reverse isolation to protect patient from infection.
5. Avoid any unnecessary invasive procedures such as venipunctures, injections, and urethral catheterizations.
I. Complementary alternative medicine
1. Arsenic trioxide (intra-venous) is an FDA-approved treatment for acute promyelocytic leukemia.
2. Follow diets that may decrease inflammation within the body such as antiyeast diets, low glycemic diet, and reduced or gluten-free diets.
3. Supplement diets to enhance immune responses against tumor growths with vitamin E, which enhances T-cell-mediated immune function, and vitamin D to help regulate the immune system response by suppressing T-cell proliferation and modulate macrophage functions.
IV. HOME CARE CONSIDERATIONS
A. Continuity of care concerns
1. The home may need to be adapted for the patient’s health needs.
2. Assistance with activities of daily living may be required during treatment or recuperation periods.
3. Home care nurses are beneficial in monitoring patient status and providing ongoing emotional support and physician liaison services.