adult T-cell leukemia-lymphoma

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adult T-cell leukemia-lymphoma

Oncology A rapidly-progressive lymphoproliferative malignancy of mature T lymphocytes, commonly associated with infection by the retrovirus, HTLV-I, first described in southeastern Japan, also seen in the Caribbean, Africa and in blacks in the southeastern US, in whom the disease is aggressive with skin lesions, hypercalcemia, rapid enlargement of hilar, retroperitoneal and peripheral lymph nodes with mediastinal sparing, invasion of CNS, lungs, GI tract, opportunistic infections–eg,.Pneumocystis carinii. See T-cell lymphoma.
Adult T-cell leukemia-lymphoma–clinical forms
Acute
Median age 52, lymphadenopathy, hepatosplenomegaly, cutaneous lesions, up to a 20-year latency, often resistant to chemotherapy with poor prognosis following disease onset Lab ↑ Ca++, WBCs 10-500 × 109–US: 10-500 000/mm3, Sezary-like cells with CD3, CD4, CD2, and Tac+ surface antigens, causing a chronic, smoldering lymphoma
Chronic
Clinically between acute and smoldering disease
Smoldering
Characterized by erythematous skin nodules filled with lymphocytes that may undergo 'blast transformation' to the typical acute T cell leukemia
Crisis
When either 2. or 3. transform to ATL
Lymphoma
Most common in US; affects blacks with hypercalcemia, leukemia, hepatosplenomegaly, erythematous skin lesions, lytic bone lesions
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