Search for a rare disease

* (*) mandatory field

Other search option(s)

Suggest an update

(*) Required fields.


Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed.

Captcha image

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.


  • 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

Detailed information


Additional information

Specialised Social Services

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.