Adult T-cell leukemia/lymphoma

Orpha code: 86875OMIM code:

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.

Disease data
Classification

Disease

Synonyms
ATLL
ATLL
ORPHA code
86875
OMIM code
-
ICD10 code
C91.5
ICD11 code
-

No additional description.

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