loading...| Preferred Name |
Aggressive NK-cell leukemia |
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| Synonyms |
NK-cell large granular lymphocyte leukemia Aggressive NK-cell lymphoma ANKCL NK-cell LGL leukemia |
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| Definitions |
An extremely rare and highly aggressive neoplasm, usually manifesting in the third to fourth decade of life, affecting males and females equally, and characterized by the onset of high fever, weight loss, jaundice, skin infiltration, lymphadenopathy, hepatosplenomegaly, and severe anemia. It has a fulminant and rapidly fatal disease course with the progressive appearance of multiorgan failure and disseminated intravascular coagulation. |
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| ID |
http://www.orpha.net/ORDO/Orphanet_86873 |
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| alternative_term |
NK-cell large granular lymphocyte leukemia Aggressive NK-cell lymphoma ANKCL NK-cell LGL leukemia |
|
| definition |
An extremely rare and highly aggressive neoplasm, usually manifesting in the third to fourth decade of life, affecting males and females equally, and characterized by the onset of high fever, weight loss, jaundice, skin infiltration, lymphadenopathy, hepatosplenomegaly, and severe anemia. It has a fulminant and rapidly fatal disease course with the progressive appearance of multiorgan failure and disseminated intravascular coagulation. |
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| definition_citation |
Orphanet |
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| expertlink |
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=en&Expert=86873 |
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| has_age_of_onset |
Adult |
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| has_inheritance |
Not applicable Multigenic/multifactorial |
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| hasDbXref |
UMLS:C1292777 ICD-10:C94.7 MedDRA:10028811 ICD-11:2A90.3 |
|
| label |
Aggressive NK-cell leukemia |
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| notation |
ORPHA:86873 |
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| part_of | ||
| prefixIRI |
ORDO:Orphanet_86873 |
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| prefLabel |
Aggressive NK-cell leukemia |
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| present_in |
Europe AND has_point_prevalence_range : Unknown |
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| treeView | ||
| subClassOf |