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