Adult T-cell leukemia/lymphoma
ORPHA:86875· ICD-10 C91.5
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.
- Prevalence
- 1-9 / 100 000
- Inheritance
- Multigenic/multifactorial, Not applicable
- Age of onset
- Adult