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

单词	adult t-cell leukemia-lymphoma
释义	DictionarySeechronicEncyclopediaSeeacute adult T-cell leukemia-lymphoma 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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