adult T-cell leukaemia-lymphoma

adult T-cell leukaemia-lymphoma

A rapidly progressive lymphoproliferative malignancy of mature T cells, typically associated with infection by the retrovirus HTLV-I.

Geography
First described in southeastern Japan, as well as the Caribbean, Africa and in African Americans in the southeastern US, in whom the disease is aggressive.

Clinical findings
Skin lesions; hypercalcemia; lytic bone lesions; multiple organ involvement; rapid enlargement of hilar; retroperitoneal and peripheral lymph nodes with mediastinal sparing; invasion of CNS, lungs and GI tract; opportunistic infections—e.g., Pneumocystis jiroveci (now carinii) or hyperinfection with Strongyloides stercoralis.

Adult T-cell leukaemia-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
Increased Ca2+, WBCs 10–500 × 109 (US): 10–500,000/mm3; Sezary-like cells with CD3, CD4, CD2 and Tac+ surface antigens, causing a chronic, smouldering lymphoma.
 
Chronic
Clinically between acute and smouldering disease.
 
Smouldering
Characterised by erythematous skin nodules filled with lymphocytes that may undergo ‘blast transformation’ to the typical acute T-cell leukaemia.
 
Crisis
When either 2 or 3 transform to ATL.
 
Lymphoma
Most common in US; affects Blacks with hypercalcemia, leukaemia, hepatosplenomegaly, erythematous skin lesions, lytic bone lesions.
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