Classic hairy cell leukemia
Synonyms: HCL-C · Leukemic reticuloendotheliosis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Unknown
- Age of onset
- Adult
- Status
- active
A rare, slowly progressive, chronic leukemia characterized by presence of abnormal B-lymphocytes (medium sized with abundant irregular pale cytoplasm, hair-like cytoplasmic projections/ruffled cytoplasmic border, a round or bean-shaped nucleus and absent nucleoli) in the blood or bone marrow, spleen and peripheral blood pancytopenia, notable monocytopenia, and marked susceptibility to infection. The characteristic immunophenotype is CD11c+, CD25+, CD103+ and CD123+ with a BRAF mutation in most cases.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Classic hairy cell leukemia | HCL-C · Leukemic reticuloendotheliosis |
| fr | Leucémie à tricholeucocytes classique | HLC-C · Réticuloendothéliose leucémique |
| de | Haarzell-Leukämie, klassische | HCL-C · HZL, klassische · Retikuloendotheliose, leukämische |
| es | Leucemia de células pilosas clásica | LCP-C · Leucemia de células peludas clásica · Reticuloendoteliosis leucémica · Tricoleucemia |
| it | Leucemia classica a cellule capellute | Reticoloendoteliosi leucemica |
| nl | Klassieke haarcelleukemie | 'Hairy cell'-leukemie · HCL-c · Leukemische reticulo-endotheliose · Pluizebolleukemie |
| pt | Leucemia de células cabeludas | Reticulo-endoteliose leucémica |
| pl | Białaczka włochatokomórkowa | HCL · Leukemic reticuloendotheliosis |
| cs | Leukémie z vlasatých buněk | VBL · Leukemická retikuloendotelióza · Vlasatobuněčná leukémie |
| uk | Класичний волосатоклітинний лейкоз | HCL-C · Лейкемічний ретикулоендотеліоз |
| zh | 经典型毛细胞白血病 | 白血病性网状内皮组织增殖 |
| tr | Klasik tüylü hücreli lösemi | HCL-C · Lösemik retiküloendotelyoz |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C91.4 | NTBT | attributed | validated |
| ICD-11 | 2A82.2 | E | specific | validated |
| MONDO | 18935 | E | validated | |
| MeSH | D007943 | E | validated | |
| UMLS | C0023443 | E | validated | |
| GARD | 6560 | E | validated | |
| MedDRA | 10019053 | 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:300842Indolent B-cell non-Hodgkin lymphoma
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 |
|---|---|---|---|---|---|---|---|---|
| BRAF | Disease-causing somatic mutation(s) in | Assessed | HGNC:1097 | ENSG00000157764 | 164757 | 7q34 | B-Raf proto-oncogene, serine/threonine kinase | 21663470[PMID]_24241536[PMID] |
Prevalence and incidence
9 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.29 / 100 000 | Europe | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Lifetime Prevalence | 1-9 / 100 000 | 3.12 / 100 000 | Europe | Value and class | Validated | 22033323[PMID]_RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.33 / 100 000 | United States | Value and class | Validated | 16150940[PMID]_National Cancer OTHERitute[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.29 / 100 000 | United Kingdom | Value and class | Validated | 7918034[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.24 / 100 000 | Israel | Value and class | Not yet validated | 17044834[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.47 / 100 000 | Iceland | Value and class | Validated | 12111650[PMID] |
| Annual incidence | <1 / 1 000 000 | 0.035 / 100 000 | Hong Kong | Value and class | Validated | 11135356[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.37 / 100 000 | Denmark | Value and class | Validated | 21122460[PMID] |
| Point prevalence | 1-9 / 100 000 | — | Europe | Class only | Not yet validated | ORPHANET |
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/58017
- This record as JSON: /data/d/58017.json