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Start free with EleplanAggressive NK-cell leukemia
ORPHA:86873Disease
Also called ANKCL · Aggressive NK-cell lymphoma · NK-cell LGL leukemia · NK-cell large granular lymphocyte leukemia
What it 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. It has a fulminant and rapidly fatal disease course with the progressive appearance of multiorgan failure and disseminated intravascular coagulation.
Key facts
- Age of onset
- Adult
- Inheritance
- Multigenic/multifactorial, Not applicable
- Classified as
- Disease
Recorded for the broader condition
- Prevalence
- <1 / 1 000 000 (annual incidence, United States)Large granular lymphocyte leukemia
Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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