adult T cell cell leukemia
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Clinical- HTLV1
- Japan, Caribbean, Central Africa
- leukemia (blood involvement)
- acute: systemic sx, rash, marked leukocytosis, hypercalcemia
- chronic: rash, minimal lymphocytosis
- smoldering: rash, normal WBC count, >5% neoplastic cells
- lymphoma (node only)
- other sites: skin, GI, marrow
Morphology- blood: flower cells (large cells with nuclear lobules)
- skin: epidermal microabscesses, dermal perivascular infiltrate
- node variants
- Hodgkin-like (RS-like B cells, background eosinophilia)
- pleomorphic small cell
- pleomorphic medium and large cells
- anaplastic large cell
Stains- positive: CD2, CD3, CD5, CD25
- can have CD30, CCR4, FOXP3
- negative: CD7, ALK
- usually CD4+, but can be CD8+ or coexpression
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