Adult T-Cell Leukemia/Lymphoma
Adult T-cell leukemia/lymphoma (ATLL) is an aggressive peripheral T-cell lymphoma caused by long-term infection with human T-lymphotropic virus 1 (HTLV-1). Patients with ATLL have an extremely poor prognosis, their disease complicated by multidrug resistance of malignant cells, infection due to impaired T-cell mediated immunity, hypercalcemia, and multiple organ failure. Half of patients develop cutaneous manifestations. The cutaneous lesions of ATLL are highly varied and include erythroderma, purpuric papules, and plaques. This chapter addresses the clinical presentation, prognosis, treatment, histopathology, immunohistochemistry, and molecular characteristics of this disease. It closes with a differential diagnosis and clinical case.
KeywordsAdult T-cell leukemia/lymphoma ATLL HTLV-1 Acute Lymphomatous Chronic Smoldering Hypercalcemia Opportunistic infection Cerebriform cells Mycosis fungoides Sézary Flower cells Viral synapse Breastfeeding Proviral integration
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