Orphanet: Leucemia ou linfoma das células T do adulto
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Adult T-cell leukemia/lymphoma

Disease definition

A rare, virus associated tumor due to human T-cell leukemia virus type 1 or human T-cell lymphotropic virus type 1 (HTLV-1) and is characterized by the presence of anti-HTLV-1 antibodies, and malignant, mature, medium-sized T cells with condensed chromatin and polylobated nuclei. The malignant cells exhibit a mature CD4+ T cells phenotype and express CD2, CD5, CD25, CD45RO, HLA-DR, and T-cell receptor αβ. Presentation is heterogeneous and is typically of aggressive leukemia or lymphoma, variable skin eruptions, and visceral organ involvement.

ORPHA:86875

Classification level: Disorder
  • Synonym(s):
    • ATLL
  • Prevalence: 1-9 / 100 000
  • Inheritance: Not applicable or Multigenic/multifactorial 
  • Age of onset: Adult
  • ICD-10: C91.5
  • OMIM: -
  • UMLS: C0023493
  • MeSH: D015459
  • GARD: -
  • MedDRA: 10001413

Detailed information

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