Chronic neutrophilic leukemia
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare myeloproliferative neoplasm characterized by sustained peripheral blood neutrophilia, bone marrow hypercellularity due to neutrophilic granulocyte proliferation, and hepatosplenomegaly. Other organs may be infiltrated in addition. Microscopically, the bone marrow shows an increase in proportion of myelocytes and mature neutrophils, but no significant dysplasia in any of the cell lineages. Peripheral blood neutrophils are mostly segmented, although band forms may also be substantially increased. Cytogenetic abnormalities are absent in most cases. The disease is slowly progressive with progredient neutrophilia followed by anemia and thrombocytopenia. Transformation to acute myeloid leukemia may occur.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Chronic neutrophilic leukemia | |
| fr | Leucémie chronique à neutrophiles | |
| de | Leukämie, chronische neutrophile | |
| es | Leucemia neutrofílica crónica | |
| it | Leucemia neutrofila cronica | |
| nl | Chronische neutrofiele leukemie | |
| pt | Leucemia neutrofílica crónica | |
| pl | Przewlekła białaczka neutrofilowa | |
| cs | Chronická neutrofilní leukémie | |
| uk | Хронічна нейтрофільна лейкемія | |
| zh | 慢性中性粒细胞白血病 | |
| tr | Kronik nötrofilik lösemi |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D47.1 | NTBT | inclusion | validated |
| ICD-11 | 2A20.1 | E | specific | validated |
| MONDO | 0019451 | E | validated | |
| MeSH | D015467 | E | validated | |
| UMLS | C0023481 | E | validated | |
| GARD | 10585 | 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:98274Myeloproliferative neoplasm
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 |
|---|---|---|---|---|---|---|---|---|
| CSF3R | Disease-causing somatic mutation(s) in | Assessed | HGNC:2439 | ENSG00000119535 | 138971 | 1p34.3 | colony stimulating factor 3 receptor | 23656643[PMID] |
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | — | Europe | Class only | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/86829
- This record as JSON: /data/d/86829.json