B-cell prolymphocytic leukemia
Synonyms: B-PLL
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adult
- Status
- active
A rare mature B-cell neoplasm characterized by clonal proliferation of B-cell prolymphocytes, with prolymphocytes constituting more than 55% of lymphoid cells in peripheral blood. IG genes are clonally rearranged. Neoplastic cells are present in the bone marrow, peripheral blood, and spleen. Patients usually present with B symptoms, massive splenomegaly but absent or minimal lymphadenopathy, rapidly increasing lymphocyte count, anemia, and thrombocytopenia. Therapy response is poor.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | B-cell prolymphocytic leukemia | B-PLL |
| fr | Leucémie prolymphocytaire à cellules B | = en |
| de | B-Zell-Prolymphozytenleukämie | B-PLL · Prolymphozytenleukämie vom B-Zell-Typ |
| es | Leucemia prolinfocítica de células B | = en |
| it | Leucemia prolinfocitica a cellule B | = en |
| nl | B-cel-prolymfocytenleukemie | = en |
| pt | Leucemia prolinfocítica de células B | |
| pl | Białaczka prolimfocytowa B-komórkowa | B-plL |
| cs | B-prolymfocytární leukémie | = en |
| uk | В-клітинна пролімфоцитарна лейкемія | = en |
| zh | B细胞幼淋巴细胞白血病 | = en |
| tr | B hücreli prolenfositik lösemi | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C91.3 | NTBT | attributed | validated |
| ICD-11 | 2A82.1 | E | specific | validated |
| MONDO | 0019461 | E | validated | |
| MeSH | D054403 | E | validated | |
| UMLS | C0475801 | E | validated | |
| GARD | 8223 | E | validated | |
| MedDRA | 10073480 | 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:300846Aggressive B-cell non-Hodgkin lymphoma
rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | 0.05 / 100 000 | Europe | Value and class | 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/86852
- This record as JSON: /data/d/86852.json