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Cutaneous Lymphomas and Sarcoma: Introducton

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  • Cutaneous lymphomas are clonal proliferations of neoplastic T or B cells, rarely natural killer cells or plasmacytoid dendritic cells. Cutaneous lymphomas are the second most common group of extranodal lymphomas. The annual incidence is estimated to be 1 per 100,000.

  • For rare conditions not dealt with in this Atlas, the reader is referred to Beyer M, Sterry W. Cutaneous lymphoma. In: Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, eds. Fitzpatrick’s Dermatology in General Medicine. 8th ed. New York, NY: McGraw-Hill; 2012:1745-1782.

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Adult T Cell Leukemia/Lymphoma

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ICD-9: 204.0/208.9 ○ ICD-10: C83/E88 Image not available.

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  • Adult T cell leukemia/lymphoma (ATLL) is a neoplasm of CD4+/CD25+ T cells, caused by human T cell lymphotrophic virus I (HTLV-I).

  • Manifested by skin infiltrates, hypercalcemia, visceral involvement, lytic bone lesions, and abnormal lymphocytes on peripheral smears.

  • HTLV-I is a human retrovirus. Infection by the virus does not usually cause disease, which suggests that other environmental factors are involved. Immortalization of some infected CD4+ T cells, increased mitotic activity, genetic instability, and impairment of cellular immunity can all occur after infection with HTLV-I. These events may increase the probability of additional genetic changes, which, by chance, may lead to the development of leukemia 20–40 years after infection in some people (≤5%). Most of these effects have been attributed to the HTLV-I-encoded protein tax.

  • ATLL occurs in southwestern Japan (Kyushu), Africa, the Caribbean Islands, and southeastern United States. Transmission is by sexual intercourse, perinatally, or by exposure to blood or blood products (same as HIV).

  • There are four main categories. In the relatively indolent smoldering and chronic forms, the median survival is ≥2 years. In the acute and lymphomatous forms, it ranges from only 4 to 6 months.

  • Symptoms include fever, weight loss, abdominal pain, diarrhea, pleural effusion, ascites, cough, and sputum. Skin lesions occur in 50% of patients with ATLL. Single to multiple small, confluent erythematous, violaceous papules (Fig. 21-1), ±purpura; firm violaceous to brownish nodules (Fig. 21-2); papulosquamous lesions, large plaques, ±ulceration; trunk > face > extremities; generalized erythroderma; poikiloderma; diffuse alopecia. Lymphadenopathy (75%) sparing mediastinal lymph nodes. Hepatomegaly (50%) and splenomegaly (25%).

  • Patients are seropositive (ELISA, Western blot) to HTLV-I; in IV drug users, up to 30% have dual retroviral infection with both HTLV-I and HIV. WBC ranges from normal to 500,000/μL. Peripheral blood smears show polylobulated lymphocytic nuclei (“flower cells”). Dermatopathology reveals lymphomatous infiltrates composed of many large abnormal lymphocytes, ±giant cells, ±Pautrier microabscesses. There is hypercalcemia–in 25% at time of diagnosis of ATLL and in >50% during clinical course; this is thought to be due to osteoclastic bone resorption.

  • Management consists of various regimens of cytotoxic chemotherapy; the rates of complete response are <30% and responses lack durability, but good results have been obtained with the combination of oral zidovudine and subcutaneous interferon-α in acute and lymphoma-type ATLL patients. Allogeneic hematopoietic stem cell transplantation holds some promise.

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Figure 21-1.

Adult T cell leukemia/lymphoma A generalized eruption of small, confluent violaceous papules with a predilection for the trunk. The patient had fever, weight loss, abdominal pain, massive leukocytosis with “flower cells” in ...

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