Mastocytosis (Q39210): Difference between revisions
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A mastocitose é devida a uma proliferação clonal neoplásica de mastócitos que se acumulam em um ou mais sistemas orgânicos. Mutações ativadoras do KIT são freqüentemente encontradas. É caracterizada pela presença de aglomerados compactos multifocais ou agregados / infiltrados coesivos de mastócitos anormais. O distúrbio é heterogêneo, variando de lesões cutâneas que podem regredir espontaneamente a neoplasias altamente agressivas associadas a falência de múltiplos órgãos e curta sobrevida. Os subtipos de mastocitose são reconhecidos principalmente pela distribuição da doença e pelas manifestações clínicas. Na mastocitose cutânea (MC), a infiltração mastocitária permanece confinada à pele, enquanto a mastocitose sistêmica (MS) é caracterizada pelo envolvimento de pelo menos um órgão extracutâneo com ou sem evidência de lesões cutâneas. A mastocitose deve ser estritamente separada da hiperplasia mastocitária ou dos estados de ativação mastocitária sem anormalidades morfológicas e / ou moleculares que caracterizam a proliferação neoplásica. | |||||||||||||||
| description / en | description / en | ||||||||||||||
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. | |||||||||||||||
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| Property / Canonical URI: https://id.who.int/icd/entity/691643472 / rank | |||||||||||||||
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CID11:2A21 | |||||||||||||||
| Property / CURIE: CID11:2A21 / rank | |||||||||||||||
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dki-india-2A21 | |||||||||||||||
| Property / Canary Token: dki-india-2A21 / rank | |||||||||||||||
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| Property / Verification Status: Concluído / rank | |||||||||||||||
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| Property / Knowledge Architect: https://pauloleads.com.br/cases-publicos/ / rank | |||||||||||||||
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13 August 2026
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| Property / Collection date: 13 August 2026 / rank | |||||||||||||||
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| Property / Linked ICD 10: C96.2 / rank | |||||||||||||||
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Latest revision as of 04:33, 13 August 2026
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.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | 2A21 |
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| English | Mastocytosis |
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. |
Statements
CID11:2A21
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dki-india-2A21
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Concluído
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13 August 2026
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