ORPHA:52416
Mantle cell lymphoma
Synonyms: LCM · MCL · Mantle zone lymphoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Multigenic/multifactorial, Not applicable
- Age of onset
- Adult
- Status
- active
Mantle cell lymphoma is a rare form of malignant non-Hodgkin lymphoma affecting B lymphocytes in the lymph nodes in a region called the ``mantle zone''.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Mantle cell lymphoma | LCM · MCL · Mantle zone lymphoma |
| fr | Lymphome à cellules du manteau | MCL |
| de | Mantelzell-Lymphom | MCL |
| es | Linfoma de células del manto | LCM · Linfoma de la zona del manto · MCL |
| it | Linfoma a cellule mantellari | LCM · Linfoma della zona mantellare · MCL |
| nl | Mantelcellymfoom | LCM · MCL · Mantelzonelymfoom |
| pt | Linfoma das células do manto | |
| pl | Chłoniak z komórek płaszcza | Chłoniak strefy brzeżnej · LCM · MCL |
| cs | Lymfom z plášťových buněk | LCM · MCL · Lymfom z buněk plášťové zóny |
| uk | Лімфома з мантійних клітин | LCM · Лімфома мантійної зони · MCL |
| zh | 套细胞淋巴瘤 | 套区淋巴瘤 |
| tr | Manto hücreli lenfoma | LCM · MCL · Mantle zon lenfoması |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C83.1 | E | specific | validated |
| ICD-11 | 2A85.5 | E | specific | validated |
| MONDO | 0018876 | E | validated | |
| MeSH | D020522 | E | validated | |
| UMLS | C4721414 | E | validated | |
| GARD | 6969 | E | validated | |
| MedDRA | 10061275 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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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.
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Genes
3 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| ATM | Disease-causing somatic mutation(s) in | Assessed | HGNC:795 | ENSG00000149311 | 607585 | 11q22.3 | ATM serine/threonine kinase | 10706620[PMID] |
| CCND1 | Disease-causing somatic mutation(s) in | Assessed | HGNC:1582 | ENSG00000110092 | 168461 | 11q13.3 | cyclin D1 | |
| IGH | Disease-causing somatic mutation(s) in | Assessed | HGNC:5477 | 146910 | 14q32.33 | immunoglobulin heavy locus | 12601901[PMID]_11245476[PMID] |
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Prevalence and incidence
3 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 100 000 | 1.05 / 100 000 | France | Value and class | Validated | OTHERitut National du Cancer 2013[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.8 / 100 000 | United States | Value and class | Validated | 28444739[PMID]_SEER Surveillance Epidemiology and End Results 2007-2013[OTHER] |
| Point prevalence | 1-9 / 100 000 | 3.5 / 100 000 | Europe | Value and class | Validated | [EXPERT]_European Medicines Agency 2015[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.
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HPO phenotypes
9 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0012191 | B-cell lymphoma | |
| HP:0002716 | Lymphadenopathy | |
| Frequent (79-30%) | ||
| HP:0005561 | Abnormality of bone marrow cell morphology | |
| HP:0002039 | Anorexia | |
| HP:0012378 | Fatigue | |
| HP:0001945 | Fever | |
| HP:0001744 | Splenomegaly | |
| HP:0001824 | Weight loss | |
| Occasional (29-5%) | ||
| HP:0011024 | Abnormality of the gastrointestinal tract | |
Run the differential on the 2 obligate / very frequent terms
Annotation source: 12548600[PMID]
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/52416
- This record as JSON: /data/d/52416.json