{"entities":{"Q39210":{"pageid":29576,"ns":120,"title":"Item:Q39210","lastrevid":118897,"modified":"2026-08-13T04:33:08Z","type":"item","id":"Q39210","labels":{"mul":{"language":"mul","value":"2A21"},"pt-br":{"language":"pt-br","value":"Mastocitose"},"en":{"language":"en","value":"Mastocytosis"}},"descriptions":{"pt-br":{"language":"pt-br","value":"A mastocitose \u00e9 devida a uma prolifera\u00e7\u00e3o clonal neopl\u00e1sica de mast\u00f3citos que se acumulam em um ou mais sistemas org\u00e2nicos. Muta\u00e7\u00f5es ativadoras do KIT s\u00e3o freq\u00fcentemente encontradas. \u00c9 caracterizada pela presen\u00e7a de aglomerados compactos multifocais ou agregados / infiltrados coesivos de mast\u00f3citos anormais. O dist\u00farbio \u00e9 heterog\u00eaneo, variando de les\u00f5es cut\u00e2neas que podem regredir espontaneamente a neoplasias altamente agressivas associadas a fal\u00eancia de m\u00faltiplos \u00f3rg\u00e3os e curta sobrevida. Os subtipos de mastocitose s\u00e3o reconhecidos principalmente pela distribui\u00e7\u00e3o da doen\u00e7a e pelas manifesta\u00e7\u00f5es cl\u00ednicas. Na mastocitose cut\u00e2nea (MC), a infiltra\u00e7\u00e3o mastocit\u00e1ria permanece confinada \u00e0 pele, enquanto a mastocitose sist\u00eamica (MS) \u00e9 caracterizada pelo envolvimento de pelo menos um \u00f3rg\u00e3o extracut\u00e2neo com ou sem evid\u00eancia de les\u00f5es cut\u00e2neas. A mastocitose deve ser estritamente separada da hiperplasia mastocit\u00e1ria ou dos estados de ativa\u00e7\u00e3o mastocit\u00e1ria sem anormalidades morfol\u00f3gicas e / ou moleculares que caracterizam a prolifera\u00e7\u00e3o neopl\u00e1sica."},"en":{"language":"en","value":"Mastocytosis is due to a clonal, neoplastic proliferation of mast cells that accumulate in one or more organ systems. Activating mutations of KIT are frequently found. It is characterised by the presence of multifocal compact clusters or cohesive aggregates/infiltrates of abnormal mast cells. The disorder is heterogeneous, ranging from skin lesions that may spontaneously regress to highly aggressive neoplasms associated with multiorgan failure and short survival. Subtypes of mastocytosis are recognised mainly by the distribution of the disease and clinical manifestations. In cutaneous mastocytosis (CM), the mast cell infiltration remains confined to the skin, whereas systemic mastocytosis (SM) is characterised by involvement of at least one extracutaneous organ with or without evidence of skin lesions. Mastocytosis should be strictly separated from mast cell hyperplasia or mast cell activation states without morphological and/or molecular abnormalities that characterize the neoplastic proliferation."}},"aliases":{},"claims":{"P68":[{"mainsnak":{"snaktype":"value","property":"P68","hash":"bf08a0afc4082dc92dec94f10c8d311a8bb6d760","datavalue":{"value":"https://id.who.int/icd/entity/691643472","type":"string"},"datatype":"url"},"type":"statement","id":"Q39210$7FA6801D-4182-461F-8629-D821B6A1C81B","rank":"normal"}],"P67":[{"mainsnak":{"snaktype":"value","property":"P67","hash":"ccc8d1e99ee6140050a97986c788c9040698a591","datavalue":{"value":"CID11:2A21","type":"string"},"datatype":"string"},"type":"statement","id":"Q39210$1D2AF57E-5042-407A-A22C-13D8CE319E39","rank":"normal"}],"P70":[{"mainsnak":{"snaktype":"value","property":"P70","hash":"612e43934342e9f663ddcd7214df09480488fce0","datavalue":{"value":"dki-india-2A21","type":"string"},"datatype":"string"},"type":"statement","id":"Q39210$9183A09A-B082-4684-A4CC-2849E1BCD48B","rank":"normal"}],"P86":[{"mainsnak":{"snaktype":"value","property":"P86","hash":"a96031634b9f5dafddaf781bbba3adea6bb64ef4","datavalue":{"value":"Conclu\u00eddo","type":"string"},"datatype":"string"},"type":"statement","id":"Q39210$52021ED7-0DAA-4055-9328-B01895DA2C2D","rank":"normal"}],"P78":[{"mainsnak":{"snaktype":"value","property":"P78","hash":"5f9b5be68b7400da549761cf15abfdbc4d1e075b","datavalue":{"value":"https://pauloleads.com.br/cases-publicos/","type":"string"},"datatype":"url"},"type":"statement","id":"Q39210$3E2AA418-1660-4457-B95A-32CA290908B5","rank":"normal"}],"P80":[{"mainsnak":{"snaktype":"value","property":"P80","hash":"92b8222fcbb75718504f915073a3f43bd4003d18","datavalue":{"value":{"time":"+2026-08-13T00:00:00Z","timezone":0,"before":0,"after":0,"precision":11,"calendarmodel":"http://www.wikidata.org/entity/Q1985727"},"type":"time"},"datatype":"time"},"type":"statement","id":"Q39210$D7BDB923-EDAB-497F-AA79-A6B43249C457","rank":"normal"}],"P89":[{"mainsnak":{"snaktype":"value","property":"P89","hash":"d3ba373476b57ef88d0d642c2c1723639d72f0ce","datavalue":{"value":{"entity-type":"item","numeric-id":13924,"id":"Q13924"},"type":"wikibase-entityid"},"datatype":"wikibase-item"},"type":"statement","id":"Q39210$B7978441-B81C-465D-AF95-8E9A3065E68F","rank":"normal"}]},"sitelinks":{}}}}