Preferred Name |
Adult T-cell leukemia/lymphoma |
|
Synonyms |
ATLL |
|
Definitions |
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. |
|
ID |
http://www.orpha.net/ORDO/Orphanet_86875 |
|
alternative_term |
ATLL |
|
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. |
|
definition_citation |
Orphanet |
|
expertlink |
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=86875 |
|
has_age_of_onset |
Adult |
|
has_inheritance |
Not applicable Multigenic/multifactorial |
|
hasDbXref |
UMLS:C0023493 ICD-10:C91.5 MeSH:D015459 ICD-11:2A90.5 MedDRA:10001413 |
|
label |
Adult T-cell leukemia/lymphoma |
|
notation |
ORPHA:86875 |
|
part_of | ||
prefixIRI |
ORDO:Orphanet_86875 |
|
prefLabel |
Adult T-cell leukemia/lymphoma |
|
present_in |
Europe AND has_point_prevalence_average_value : 3.0 AND has_point_prevalence_range : 1-9 / 100 000 |
|
treeView | ||
subClassOf |