Updated: 12/18/2017

Adult T-Cell Leukemia / Lymphoma (ATLL)

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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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