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716655008: Aggressive systemic mastocytosis (disorder)

Status: current, Sufficiently defined by necessary conditions definition status (core metadata concept). Date: 31-Jul 2021. Module: SNOMED CT core

Descriptions:

Id Description Lang Type Status Case? Module
3307099016 Aggressive systemic mastocytosis (disorder) en Fully specified name Active Entire term case insensitive (core metadata concept) SNOMED CT core
3307100012 Aggressive systemic mastocytosis en Synonym (core metadata concept) Active Entire term case insensitive (core metadata concept) SNOMED CT core
5401344010 A rare, aggressive form of advanced systemic mastocytosis (advSM) characterized by massive infiltration of mast cells (MC) in different tissues and presence of extracutaneous organ dysfunction, but without evidence of mast cell leukemia or another hematologic neoplasm. en Definition Active Entire term case sensitive (core metadata concept) SNOMED CT core
5401345011 A rare, aggressive form of advanced systemic mastocytosis (advSM) characterised by massive infiltration of mast cells (MC) in different tissues and presence of extracutaneous organ dysfunction, but without evidence of mast cell leukaemia or another haematologic neoplasm. en Definition Active Entire term case sensitive (core metadata concept) SNOMED CT core
3307099016 Aggressive systemic mastocytosis (disorder) en Fully specified name Active Only initial character case insensitive (core metadata concept) SNOMED CT core
3307099016 Aggressive systemic mastocytosis (disorder) en Fully specified name Active Entire term case insensitive (core metadata concept) SNOMED CT core
3307100012 Aggressive systemic mastocytosis en Synonym (core metadata concept) Active Only initial character case insensitive (core metadata concept) SNOMED CT core
3307100012 Aggressive systemic mastocytosis en Synonym (core metadata concept) Active Entire term case insensitive (core metadata concept) SNOMED CT core
3307101011 A severe and rare form of systemic mastocytosis with manifestation of considerable infiltration of mast cells in different tissues. It represents less than 10% of cases of systemic mastocytosis. This disease doesn't usually develop in children. Cutaneous involvement is normally absent. Patients present with severe symptoms related to mast cell invasion and the intense release of mediators including syncope, recurrent flushing, organomegaly and organ dysfunction. The most serious complication is potentially fatal anaphylactic shock.There is evidence of an activating mutation of KIT, usually D816V, in the mast cells of the vast majority of patients. The prognosis is poor with a median survival of 2 to 4 years. en Definition Active Entire term case sensitive (core metadata concept) SNOMED CT core
5401344010 A rare, aggressive form of advanced systemic mastocytosis (advSM) characterized by massive infiltration of mast cells (MC) in different tissues and presence of extracutaneous organ dysfunction, but without evidence of mast cell leukemia or another hematologic neoplasm. en Definition Active Entire term case sensitive (core metadata concept) SNOMED CT core
5401345011 A rare, aggressive form of advanced systemic mastocytosis (advSM) characterised by massive infiltration of mast cells (MC) in different tissues and presence of extracutaneous organ dysfunction, but without evidence of mast cell leukaemia or another haematologic neoplasm. en Definition Active Entire term case sensitive (core metadata concept) SNOMED CT core
3411161001000115 Mastzell-Leukämie, agressive de Synonym (core metadata concept) Active Entire term case sensitive (core metadata concept) SNOMED CT Switzerland NRC maintained Module
964471000172110 mastocytose systémique agressive fr Synonym (core metadata concept) Active Entire term case insensitive (core metadata concept) SNOMED CT Switzerland NRC maintained Module
964471000172110 mastocytose systémique agressive fr Synonym (core metadata concept) Active Entire term case insensitive (core metadata concept) SNOMED CT Switzerland NRC maintained Module
3411161001000115 Mastzell-Leukämie, agressive de Synonym (core metadata concept) Active Entire term case sensitive (core metadata concept) SNOMED CT Switzerland NRC maintained Module

0 descendants.

Expanded Value Set

Outbound Relationships Type Target Active Characteristic Refinability Group Values
Aggressive systemic mastocytosis (disorder) Associated morphology Mast cell abnormality (morphologic abnormality) false Inferred relationship Existential restriction modifier (core metadata concept) 2
Aggressive systemic mastocytosis (disorder) Is a Systemic mast cell disease (disorder) false Inferred relationship Existential restriction modifier (core metadata concept)
Aggressive systemic mastocytosis (disorder) Has definitional manifestation Immune system finding false Inferred relationship Existential restriction modifier (core metadata concept)
Aggressive systemic mastocytosis (disorder) Pathological process (attribute) Abnormal immune process (qualifier value) false Inferred relationship Existential restriction modifier (core metadata concept) 1
Aggressive systemic mastocytosis (disorder) Associated morphology Hyperplasia false Inferred relationship Existential restriction modifier (core metadata concept) 2
Aggressive systemic mastocytosis (disorder) Finding site Mast cell false Inferred relationship Existential restriction modifier (core metadata concept) 2
Aggressive systemic mastocytosis (disorder) Pathological process (attribute) Abnormal immune process (qualifier value) false Inferred relationship Existential restriction modifier (core metadata concept) 1
Aggressive systemic mastocytosis (disorder) Associated morphology Hyperplasia false Inferred relationship Existential restriction modifier (core metadata concept) 1
Aggressive systemic mastocytosis (disorder) Finding site Mast cell false Inferred relationship Existential restriction modifier (core metadata concept) 1
Aggressive systemic mastocytosis (disorder) Associated morphology Aggressive systemic mastocytosis true Inferred relationship Existential restriction modifier (core metadata concept) 1
Aggressive systemic mastocytosis (disorder) Is a mastocytose maligne true Inferred relationship Existential restriction modifier (core metadata concept)
Inbound Relationships Type Active Source Characteristic Refinability Group

Reference Sets

Component annotation with string value reference set (foundation metadata concept)

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