ORPHA:52417
MALT lymphoma
Synonyms: Extranodal marginal zone B-cell lymphoma · MALToma · Mucosa-associated lymphatic tissue lymphoma · Mucosa-associated lymphoid tissue lymphoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Multigenic/multifactorial, Not applicable
- Age of onset
- Adult
- Status
- active
MALT (mucosa-associated lymphoid tissue) lymphoma is a rare form of malignant non-Hodgkin lymphoma that affects B cells and grows at the expense of lymphoid tissue associated with mucous membranes, but also occurs, more rarely, in lymph nodes.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | MALT lymphoma | Extranodal marginal zone B-cell lymphoma · MALToma · Mucosa-associated lymphatic tissue lymphoma · Mucosa-associated lymphoid tissue lymphoma |
| fr | Lymphome MALT | Lymphome du tissu lymphoïde associé aux muqueuses · Lymphome extraganglionnaire de la zone marginale |
| de | MALT-Lymphom | Lymphom des mukosa-assoziierten lymphatischen Gewebes · MALTom · Marginalzonen-B-Zell-Lymphom vom MALT-Typ · Marginalzonen-Lymphom, extranodales |
| es | Linfoma MALT | Linfoma de tejido linfoide asociado a mucosa · Linfoma de tejido linfático asociado a mucosa · Linfoma extranodal de zona marginal · MALToma |
| it | Linfoma MALT | Linfoma del tessuto linfatico associato alle mucose · Linfoma del tessuto linfoide associato alle mucose · Linfoma della zona marginale extranodale · MALToma |
| nl | MALT-lymfoom | Extranodale marginale zone B-cellymfoom · MALToom · Mucosageassocieerd lymfatisch weefsellymfoom · Mucosageassocieerd lymfoïd weefsellymfoom |
| pt | Linfoma MALT | |
| pl | Chłoniak MALT | Chłoniak tkanki limfatycznej związany z błoną śluzową · MALToma · Pozawęzłowy chłoniak strefy brzeżnej z komórek B |
| cs | MALT lymfom | Extranodální B-lymfom z marginální zóny · MALTom · Lymfom z lymfatické tkáně se vztahem ke sliznicím |
| uk | Лімфома MALT | Екстранодальна В-клітинна лімфома маргінальної зони · MALToma · Слизово-асоційована лімфома лімфатичної тканини · Лімфома лімфоїдної тканини, асоційована зі слизовою оболонкою |
| zh | MALT淋巴瘤 | 黏膜相关淋巴组织淋巴瘤 |
| tr | MALT lenfoma | MALToma · Ekstranodal marjinal bölge B hücreli lenfoma · Mukozayla ilişkili lenfoid doku lenfoması · Mukozayla ilişkili lenfatik doku lenfoması |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 137245 | E | validated | |
| ICD-10 | C88.4 | E | specific | validated |
| MONDO | 0007650 | E | validated | |
| UMLS | C1850900 | E | validated | |
| GARD | 6485 | E | validated | |
| MedDRA | 10060707 | 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 (2):
- ORPHA:300912Marginal zone lymphoma
rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders - ORPHA:717877Rare miscellaneous disease with inflammatory bowel disease
rare gastroenterological diseases; rare genetic diseases; rare transplant-related disorders
No children.
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Genes
5 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| BCL10 | Part of a fusion gene in | Assessed | HGNC:989 | ENSG00000142867 | 603517 | 1p22.3 | BCL10 immune signaling adaptor | 25922601[PMID]_10319863[PMID] |
| BIRC3 | Part of a fusion gene in | Assessed | HGNC:591 | ENSG00000023445 | 601721 | 11q22.2 | baculoviral IAP repeat containing 3 | |
| FOXP1 | Part of a fusion gene in | Assessed | HGNC:3823 | ENSG00000114861 | 605515 | 3p13 | forkhead box P1 | 15703784[PMID] |
| IGH | Part of a fusion gene in | Assessed | HGNC:5477 | 146910 | 14q32.33 | immunoglobulin heavy locus | 15703784[PMID] | |
| MALT1 | Part of a fusion gene in | Assessed | HGNC:6819 | ENSG00000172175 | 604860 | 18q21.32 | MALT1 paracaspase |
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Prevalence and incidence
3 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.3 / 100 000 | Europe | Value and class | Validated | [EXPERT]_RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 1.59 / 100 000 | United States | Value and class | Validated | 22893605[PMID]_National Cancer OTHERitute[OTHER] |
| Point prevalence | 1-9 / 100 000 | 4 / 100 000 | Europe | Value and class | Validated | European Medicines Agency 2013[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
17 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0001903 | Anemia | |
| HP:0012191 | B-cell lymphoma | |
| HP:0012378 | Fatigue | |
| HP:0001945 | Fever | |
| HP:0000975 | Hyperhidrosis | |
| HP:0002017 | Nausea and vomiting | |
| HP:0002113 | Pulmonary infiltrates | |
| HP:0001824 | Weight loss | |
| Frequent (79-30%) | ||
| HP:0002019 | Constipation | |
| Occasional (29-5%) | ||
| HP:0002027 | Abdominal pain | |
| HP:0000614 | Abnormal nasolacrimal system morphology | |
| HP:0000820 | Abnormality of the thyroid gland | |
| HP:0002716 | Lymphadenopathy | |
| HP:0100721 | Mediastinal lymphadenopathy | |
| HP:0012123 | Posterior uveitis | |
| HP:0002205 | Recurrent respiratory infections | |
| HP:0000505 | Visual impairment | |
Run the differential on the 8 obligate / very frequent terms
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/52417
- This record as JSON: /data/d/52417.json