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Updated: Dec 18 2017

Adult T-Cell Leukemia / Lymphoma (ATLL)

  • Snapshot
    • A 50-year-old man originally from the Carribean islands complains of weakness and chronic constipation. He has an erythematous rash covering > 90% of his body surface area, with well-demarcated papules as well as purpuric lesions. He is also found with hepatosplenomegaly and lymphadenopathy. A peripheral blood smear reveals “flower” cells.
  • Introduction
    • ATLL is an aggressive mature T-cell malignancy
      • a type of cutaneous T-cell lymphoma
    • Pathogenesis
      • human T-cell lymphotrophic virus (HTLV-1)
    • Epidemiology
      • endemic regions
        • Japan
        • Caribbean
        • sub-Saharan Africa
        • Middle East
        • Central and South America
    • Characteristic finding is “flower” cell on peripheral blood smear
  • Presentation
    • Symptoms
      • hypercalcemia
        • constipation
        • altered mental status
        • kidney stone
        • weakness
      • fatigue
      • anorexia
    • Physical exam
      • hepatoseplenomegaly in 50%
      • lymphadenopathy in almost all
      • skin lesions
        • variable morphology
        • plaque, papule, nodulotumoral, erythroderma, and purpuric
  • Evaluation
    • Complete blood count > 5% abnormal lymphocytes
    • Peripheral blood smear with “flower” cells
    • Serology
      • antibodies to HTLV-1
    • Hypercalcemia
    • Flow cytometry or immunohistochemistry
      • CD4+, CD25+, CD52+, CD7-, and CD8-
    • Imaging for lytic lesions
  • Differential Diagnosis
    • Mycosis fungoides
    • Sézary syndrome
    • Cutaneous metastases of other malignancy
  • Treatment
    • Zidovudine
    • INFα
    • Chemotherapy
  • Prognosis, Prevention, and Complications

    • Prognosis
      • poor
      • median survival time 6 months - 2 years
    • Prevention
      • to prevent mother to infant transmission
        • cessation of breastfeeding in moms with known HTLV-1
      • avoid sexual contact with infected individuals
    • Complications
      • opportunistic infections
      • lytic bone lesions
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