{"orpha":52417,"name":"MALT lymphoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Extranodal marginal zone B-cell lymphoma","MALToma","Mucosa-associated lymphatic tissue lymphoma","Mucosa-associated lymphoid tissue lymphoma"],"definition":"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.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-52417/","orphanet_url":"https://www.orpha.net/en/disease/detail/52417","names":{"fr":{"name":"Lymphome MALT","synonyms":["Lymphome du tissu lymphoïde associé aux muqueuses","Lymphome extraganglionnaire de la zone marginale"],"definition":"Le lymphome de type MALT (mucosa-associated lymphoid tissue) est une forme rare de lymphome malin non-Hodgkinien qui affecte les lymphocytes B et se développe aux dépens du tissu lymphoïde associé aux muqueuses, mais aussi de manière plus rare au niveau d'un ganglion."},"de":{"name":"MALT-Lymphom","synonyms":["Lymphom des mukosa-assoziierten lymphatischen Gewebes","MALTom","Marginalzonen-B-Zell-Lymphom vom MALT-Typ","Marginalzonen-Lymphom, extranodales"],"definition":"Das MALT(mucosa-associated lymphoid tissue)-Lymphom ist eine seltene Form des malignen Non-Hodgkin-Lymphoms (s. dort). Es betrifft die B-Zellen und wächst auf Kosten des lymphoiden Gewebes der Schleimhäute. Seltener tritt es auch in den Lymphknoten auf. Es macht etwa 5% der jährlich diagnostizieren Fälle von Non-Hodgkin-Lymphom aus."},"es":{"name":"Linfoma MALT","synonyms":["Linfoma de tejido linfoide asociado a mucosa","Linfoma de tejido linfático asociado a mucosa","Linfoma extranodal de zona marginal","MALToma"],"definition":"El linfoma MALT (linfoma de tejido linfoide asociado a mucosas) es una forma rara del linfoma maligno no Hodgkin (ver este término), que afecta a las células B y que se desarrolla a expensas del tejido linfoide asociado a las membranas mucosas, aunque también ocurre, más raramente, en los ganglios linfáticos."},"it":{"name":"Linfoma MALT","synonyms":["Linfoma del tessuto linfatico associato alle mucose","Linfoma del tessuto linfoide associato alle mucose","Linfoma della zona marginale extranodale","MALToma"]},"nl":{"name":"MALT-lymfoom","synonyms":["Extranodale marginale zone B-cellymfoom","MALToom","Mucosageassocieerd lymfatisch weefsellymfoom","Mucosageassocieerd lymfoïd weefsellymfoom"],"definition":"Een zeldzame vorm van maligne non-Hodgkin-lymfoom, die B-cellen aantast en die groeit ten koste van lymfoïd weefsel geassocieerd met slijmvlies, maar in zeldzamere gevallen ook optreedt in lymfeklieren."},"pt":{"name":"Linfoma MALT"},"pl":{"name":"Chłoniak MALT","synonyms":["Chłoniak tkanki limfatycznej związany z błoną śluzową","MALToma","Pozawęzłowy chłoniak strefy brzeżnej z komórek B"]},"cs":{"name":"MALT lymfom","synonyms":["Extranodální B-lymfom z marginální zóny","MALTom","Lymfom z lymfatické tkáně se vztahem ke sliznicím"]},"uk":{"name":"Лімфома MALT","synonyms":["Екстранодальна В-клітинна лімфома маргінальної зони","MALToma","Слизово-асоційована лімфома лімфатичної тканини","Лімфома лімфоїдної тканини, асоційована зі слизовою оболонкою"],"definition":"MALT (mucosa-associated lymphoid tissue) lymphoma is a rare form of malignant non-Hodgkin lymphoma (see this term) that affects B cells and grows at the expense of lymphoid tissue associated with mucous membranes, but also occurs, more rarely, in lymph nodes."},"zh":{"name":"MALT淋巴瘤","synonyms":["黏膜相关淋巴组织淋巴瘤"]},"tr":{"name":"MALT lenfoma","synonyms":["MALToma","Ekstranodal marjinal bölge B hücreli lenfoma","Mukozayla ilişkili lenfoid doku lenfoması","Mukozayla ilişkili lenfatik doku lenfoması"]}},"xrefs":[{"source":"OMIM","code":"137245","qualifier":"E","icd_relation":null,"validated":true},{"source":"ICD-10","code":"C88.4","qualifier":"E","icd_relation":"specific","validated":true},{"source":"MONDO","code":"0007650","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1850900","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"6485","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10060707","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":300912,"name":"Marginal zone lymphoma","classifications":[194,202,233]},{"orpha":717877,"name":"Rare miscellaneous disease with inflammatory bowel disease","classifications":[152,156,233]}],"children":[],"genes":[{"symbol":"BCL10","name":"BCL10 immune signaling adaptor","gene_type":"gene with protein product","association":"Part of a fusion gene in","status":"Assessed","source":"25922601[PMID]_10319863[PMID]","hgnc":"989","ensembl":"ENSG00000142867","omim":"603517","loci":["1p22.3"]},{"symbol":"BIRC3","name":"baculoviral IAP repeat containing 3","gene_type":"gene with protein product","association":"Part of a fusion gene in","status":"Assessed","source":null,"hgnc":"591","ensembl":"ENSG00000023445","omim":"601721","loci":["11q22.2"]},{"symbol":"FOXP1","name":"forkhead box P1","gene_type":"gene with protein product","association":"Part of a fusion gene in","status":"Assessed","source":"15703784[PMID]","hgnc":"3823","ensembl":"ENSG00000114861","omim":"605515","loci":["3p13"]},{"symbol":"IGH","name":"immunoglobulin heavy locus","gene_type":"gene with protein product","association":"Part of a fusion gene in","status":"Assessed","source":"15703784[PMID]","hgnc":"5477","ensembl":null,"omim":"146910","loci":["14q32.33"]},{"symbol":"MALT1","name":"MALT1 paracaspase","gene_type":"gene with protein product","association":"Part of a fusion gene in","status":"Assessed","source":null,"hgnc":"6819","ensembl":"ENSG00000172175","omim":"604860","loci":["18q21.32"]}],"genes_via_subtypes":[],"inheritance":["Multigenic/multifactorial","Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.3,"area":"Europe","status":"Validated","source":"[EXPERT]_RARECARE surveillance of rare cancers in Europe[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":1.59,"area":"United States","status":"Validated","source":"22893605[PMID]_National Cancer OTHERitute[OTHER]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":4.0,"area":"Europe","status":"Validated","source":"European Medicines Agency 2013[OTHER]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-06-01","source":null,"terms":[{"hpo":"HP:0001903","label":"Anemia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012191","label":"B-cell lymphoma","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012378","label":"Fatigue","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000975","label":"Hyperhidrosis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002017","label":"Nausea and vomiting","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002113","label":"Pulmonary infiltrates","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001824","label":"Weight loss","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002019","label":"Constipation","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002027","label":"Abdominal pain","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000614","label":"Abnormal nasolacrimal system morphology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000820","label":"Abnormality of the thyroid gland","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002716","label":"Lymphadenopathy","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100721","label":"Mediastinal lymphadenopathy","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012123","label":"Posterior uveitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002205","label":"Recurrent respiratory infections","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000505","label":"Visual impairment","frequency":"Occasional (29-5%)","criteria":null}]}}