716655008: Aggressive systemic mastocytosis (disorder)

• SNOMED CT Concept\Clinical finding (finding)\Disease\...
• \Disorder of hematopoietic cell proliferation (disorder)\Mast cell neoplasm (disorder)\...
• \Systemic mast cell disease (disorder)\Malignant mastocytosis\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.
• \Mast cell malignancy (disorder)\Malignant mastocytosis\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.
• \Neoplasm and/or hamartoma (disorder)\Neoplastic disease\...
• \Neoplasm of hematopoietic cell type (disorder)\Mast cell neoplasm (disorder)\...
• \Systemic mast cell disease (disorder)\Malignant mastocytosis\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.
• \Mast cell malignancy (disorder)\Malignant mastocytosis\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.
• \Malignant neoplastic disease (disorder)\Mast cell malignancy (disorder)\Malignant mastocytosis\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.
• \Systemic disease (disorder)\Systemic mast cell disease (disorder)\Malignant mastocytosis\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.

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
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	Inactive	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
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

Expanded Value Set

Reference Sets

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

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