Orphanet Rare Disease Ontology

Last uploaded: December 13, 2023
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

http://www.orpha.net/ORDO/Orphanet_178551

http://www.orpha.net/ORDO/Orphanet_289635

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

http://www.orpha.net/ORDO/Orphanet_178551

http://www.orpha.net/ORDO/Orphanet_289635

subClassOf

http://www.orpha.net/ORDO/Orphanet_377788

http://www.orpha.net/ORDO/Orphanet_557493

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