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716655008: Aggressive systemic mastocytosis (disorder)

Status: current, Defined. 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 Case insensitive SNOMED CT core
3307100012 Aggressive systemic mastocytosis en Synonym Active Case insensitive 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 Case sensitive SNOMED CT core

0 descendants.

Expanded Value Set

Outbound Relationships Type Target Active Characteristic Refinability Group Values
Aggressive systemic mastocytosis Pathological process Abnormal immune process false Inferred relationship Some 1
Aggressive systemic mastocytosis Associated morphology Hyperplasia false Inferred relationship Some 2
Aggressive systemic mastocytosis Finding site Mast cell false Inferred relationship Some 2
Aggressive systemic mastocytosis Pathological process Abnormal immune process false Inferred relationship Some 1
Aggressive systemic mastocytosis Associated morphology Hyperplasia false Inferred relationship Some 1
Aggressive systemic mastocytosis Finding site Mast cell false Inferred relationship Some 1
Aggressive systemic mastocytosis Associated morphology Aggressive systemic mastocytosis true Inferred relationship Some 1
Aggressive systemic mastocytosis Is a Malignant mastocytosis true Inferred relationship Some
Aggressive systemic mastocytosis Associated morphology Mast cell abnormality false Inferred relationship Some 2
Aggressive systemic mastocytosis Is a Systemic mast cell disease false Inferred relationship Some
Aggressive systemic mastocytosis Has definitional manifestation Immune system finding false Inferred relationship Some
Inbound Relationships Type Active Source Characteristic Refinability Group

Reference Sets

Australian emergency department reference set

Clinical finding foundation reference set

Neoplasm and/or hamartoma reference set

Problem/Diagnosis reference set

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