Chronic lymphoproliferative disorder of natural killer cells
Synonyms: CLPD-NK · CNKL · Chronic NK lymphocytosis · Chronic NK-cell lymphocytosis · Chronic lymphoproliferative disorder of NK-cells · NK-cell lineage granular lymphocyte proliferative disorder
- Type
- Disease
- Group
- Disorder
- Inheritance
- Unknown
- Status
- active
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.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Chronic lymphoproliferative disorder of natural killer cells | CLPD-NK · CNKL · Chronic NK lymphocytosis · Chronic NK-cell lymphocytosis · Chronic lymphoproliferative disorder of NK-cells · NK-cell lineage granular lymphocyte proliferative disorder |
| fr | Leucémie chronique à grands lymphocytes granuleux à cellules NK | Leucémie chronique à GLG-NK · Leucémie chronique à LGL-NK · Leucémie lymphoïde à grands lymphocytes granuleux de type NK · Lymphocytose GLG à cellules NK · Lymphocytose LGL à cellules NK · Syndrome lymphoprolifératif chronique à cellules NK |
| de | Chronische lymphoproliferative Krankheit der natürlichen Killer-Zellen | CLPD-NK · Chronische NK-Zellen-Lymphozytose · Chronische lymphoproliferative Krankheit der NK-Zellen · Proliferative Krankheit granulärer Lymphozyten der NK-Zelllinie |
| es | Trastorno linfoproliferativo crónico de células natural killer | CLPD-NK · CNKL · Linfocitosis crónica NK · Linfocitosis crónica de células NK · Trastorno linfoproliferativo crónico de células NK · Trastorno proliferativo de linfocitos granulares de línea de células NK |
| it | Malattia linfoproliferativa cronica delle cellule natural killer | CLPD-NK · CNKL · Disordine linfoproliferativo cronico delle cellule NK · Disordine proliferativo dei linfociti granulari della linea delle cellule NK · Linfocitosi cronica delle cellule NK · NK linfocitosi cronica |
| nl | Chronische lymfoproliferatieve stoornis van natuurlijke killercellen | CLPD-NK · CNKL · Chronische NK-cellymfocytose · Chronische NK-lymfocytose · Chronische lymfoproliferatieve aandoening van NK-cellen · Proliferatieve aandoening van granulaire lymfocyten van NK-cellijn |
| pt | Doença linfoproliferativa crónica das célualas NK | |
| pl | Przewlekła choroba limfoproliferacyjna z limfocytów typu "naturalni zabójcy | CNKL · CLPD-NK · Przewlekła limfocytoza NK · Przewlekła limfocytoza z komórek NK · Przewlekła choroba limfoproliferacyjna z komórek NK · Choroba proliferacyjna limfocytów ziarnistych z linii NK |
| cs | Chronická proliferativní porucha granulárních lymfocytů z NK buněčné linie | CNKL · CLPD-NK · Chronická lymfocytóza NK buněk · Chronická lymfoproliferativní porucha NK buněk · Proliferativní porucha granulárních lymfocytů z NK buněčné linie |
| uk | Хронічне лімфопроліферативне захворювання природних кілерів | Хронічне лімфопроліферативне захворювання NK-клітин · Хронічний NK-лімфоцитоз · Хронічний NK-клітинний лімфоцитоз · CLPD-NK · CNKL · Порушення проліферації гранулярних лімфоцитів NK-клітинної лінії |
| zh | 慢性自然杀伤细胞性淋巴增殖障碍 | 自然杀伤细胞谱系性颗粒淋巴增殖障碍 |
| tr | Doğal öldürücü hücrelerin kronik lenfoproliferatif bozukluğu | CNKL · CLPD-NK · Kronik NK lenfositoz · Kronik NK hücreli lenfositoz · NK hücrelerinin kronik lenfoproliferatif bozukluğu · NK hücre soyu granüler lenfosit proliferatif bozukluğu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C91.7 | NTBT | attributed | validated |
| ICD-11 | 2A90.2 | E | specific | validated |
| MONDO | 0004234 | E | validated | |
| UMLS | C1512709 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:512034Large granular lymphocyte leukemia
rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders
No children.
Genes
1 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| STAT3 | Disease-causing germline mutation(s) in | Assessed | HGNC:11364 | ENSG00000168610 | 102582 | 17q21.2 | signal transducer and activator of transcription 3 | 29385279[PMID] |
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/512017
- This record as JSON: /data/d/512017.json