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Aggressive NK-cell leukemia

Disease definition

Aggressive NK-cell leukemia (ANKCL) is 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. ANKCL has a fulminant and rapidly fatal disease course with the progressive appearance of multiorgan failure and disseminated intravascular coagulation.

ORPHA:86873

  • Synonym(s):
    • ANKCL
    • Aggressive NK-cell lymphoma
    • NK-cell LGL leukemia
    • NK-cell large granular lymphocyte leukemia
  • Prevalence: Unknown
  • Inheritance: Not applicable or Multigenic/multifactorial 
  • Age of onset: Adult
  • ICD-10: C94.7
  • OMIM: -
  • UMLS: C1292777  C1522378
  • MeSH: -
  • GARD: 10493
  • MedDRA: 10028811

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