maligne mastocytose
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agressieve systemische mastocytose (aandoening)
agressieve systemische mastocytose
agressieve systemische mestcelziekte
Aggressive systemic mastocytosis
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.
Id716655008
StatusDefined
Associated morphologyagressieve systemische mastocytose
DHD Diagnosis thesaurus reference set
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set
TargetC96.2
TermMestceltumor, maligne
SNOMED CT to Orphanet simple map98850
SNOMED CT to ICD-10 extended map
TargetC96.2
RuleTRUE
AdviceALWAYS C96.2 | POSSIBLE REQUIREMENT FOR MORPHOLOGY CODE
CorrelationSNOMED CT source code to target map code correlation not specified