Adult T-cell leukemia/lymphoma

    Article Contributors: 
    Sean Klepper M.D.
    Artur Zembowicz M.D....

    Clinical Features:

    • An aggressive lymphoproliferative disorder caused by infection with the virus HTLV-1, which is endemic in Japan, the Caribbean, South America and central Africa
    • The lifeteme risk of developing ATLL among individuals infected with HTLV-1 is 2.5%.
    • Four clinical forms: acute, chronic, smoldering and lymphoma
    • The acute form presents with high-grade leukemia, marrow involvement, skin lesions, generalized lymphadenopathy ane hepatosplenomegaly.
    • Characteristic of all forms is hypercalcemia, extreme thirst and circulating CD25 (IL-2 receptor; HTLV-1 infects CD4+ T-cells by way of this receptor)
    • Diagnosis is based upon clinical features with serologic demonstration of anti-HTLV-1 antibodies.
    • Cutaneous manifestations are variable, and can include generalized erythroderma, a maculopapular rash, nodules, plaques and bullous lesions.

    Histologic Features:

    • Dense lymphoid infiltrate throughout the dermis and subcutaneous fat
    • The infiltrate is often most dense around blood vessels and skin appendages.
    • Exocytosis into the epidermis, with or without Pautrier's microabscesses, often occurs.
    • The neoplastic lymphocytes range in size from small to large and have markedly convoluted nuclei.
    • There is often marked dermal edema.
    • Vasculitis with red blood cell extravasation is often seen.
    • Circulating leukemic cells have large convoluted nuclei said to resemble a flower with petals ("flower cells")
    • Immunophenotypically, the cells are helper T-cells (CD3+, CD4+, CD5+, CD8+), usually with loss of CD7 and characteristic expression of CD25.
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