Plasma cell leukemia
Synonyms: PCL
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly
- Status
- active
A rare plasma cell neoplasm characterized by peripheral plasmacytosis, usually with extensive and diffuse infiltration of the bone marrow, and monoclonal paraproteinemia. Neoplastic plasma cells may also be found in extramedullary sites, such as the liver or spleen, among others. Most cases present as primary plasma cell leukemia without previous diagnosis of myeloma. The condition can also represent leukemic transformation of plasma cell myeloma (secondary plasma cell leukemia). Clinical manifestations include lymphadenopathy, organomegaly, renal failure, bone marrow failure, and peripheral neuropathies. High serum levels of lactate dehydrogenase and beta2-microglobulin, as well as hypercalcemia (potentially leading to hypercalcemic crisis) are typically observed.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Plasma cell leukemia | PCL |
| fr | Leucémie à plasmocytes | |
| de | Plasmazell-Leukämie | = en |
| es | Leucemia de células plasmáticas | LCP |
| it | Leucemia plasmacellulare | = en |
| nl | Plasmacelleukemie | = en |
| pt | Leucemia por plasmócitos | |
| pl | Białaczka plazmocytowa | = en |
| cs | Plazmocelulární leukémie | = en |
| uk | Плазмоклітинна лейкемія | = en |
| zh | 浆细胞白血病 | = en |
| tr | Plazma hücreli lösemi | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C90.1 | E | specific | validated |
| ICD-11 | 2A83.4 | E | specific | validated |
| MONDO | 0018689 | E | validated | |
| MeSH | D007952 | E | validated | |
| UMLS | C0023484 | E | validated | |
| GARD | 9373 | E | validated | |
| MedDRA | 10035222 | 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:98282Plasma cell tumor
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 | 0.04 / 100 000 | Europe | Value and class | Validated | 20664057[PMID] |
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/454714
- This record as JSON: /data/d/454714.json