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CUTANEOUS LYMPHOMAS AND SARCOMA: INTRODUCTION

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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.

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ADULT T CELL LEUKEMIA/LYMPHOMA ICD-10: C83/E88

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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.

  • ATLL occurs in southwestern Japan (Kyushu), Africa, the Caribbean Islands, and the 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 caused by 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 has shown 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 smear, lymphadenopathy, hepatosplenomegaly, and hypercalcemia.

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Figure 21-2

Adult T cell leukemia/lymphoma Firm, violaceous to brownish nodules as shown here are another cutaneous manifestation of ATLL. These nodules may ulcerate.

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CUTANEOUS T CELL LYMPHOMA ICD-10: C84.0/C84.1

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  • Cutaneous T cell lymphoma (CTCL) is a term that applies to T cell lymphoma first manifested in the ...

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