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Start free with EleplanChronic lymphoproliferative disorder of natural killer cells
ORPHA:512017Disease
Also called CLPD-NK · CNKL · Chronic NK lymphocytosis · Chronic NK-cell lymphocytosis · Chronic lymphoproliferative disorder of NK-cells · NK-cell lineage granular lymphocyte proliferative disorder
What it is
A rare large granular lymphocyte leukemia characterized by persistent (> 6 months) natural killer cell lymphocytosis in the absence of clinical diagnosis of leukemia/lymphoma, autoimmune disease, or chronic viral infections. The clinical course is variable, but generally indolent. Patients often remain asymptomatic, or may present with clinical manifestations including vasculitic skin lesions, neutropenic infections, musculoskeletal symptoms, peripheral neuropathy, or splenomegaly.
Key facts
- Inheritance
- Unknown
- 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.
Gene
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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