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monoklonaal mestcelactivatiesyndroom (aandoening)
monoklonaal mestcelactivatiesyndroom
monoklonaal MCAS
Monoclonal mast cell activation syndrome
Monoclonal MCAD (mast cell activation disorder)
A rare hematologic disease characterized by symptoms of mast cell activation in the absence of cutaneous findings, as well as absence of diagnostic criteria of systemic mastocytosis with tryptase levels of less than 20 mg/ml and normal to low burden of mast cells. Bone marrow biopsy reveals the presence of monoclonal mast cells carrying the KIT D816V mutation and/or expressing CD25. Symptoms include recurrent episodes of flushing, headache, hypotension, abdominal cramping, nausea, diarrhea, cardiac arrhythmia, bronchoconstriction and bleeding diathesis.
Id1260202003
StatusPrimitive
Finding sitemastocytus
Pathological processafwijkend immuunproces
SNOMED CT to Orphanet simple map529468
SNOMED CT to ICD-10 extended map
TargetC94.3
RuleTRUE
AdviceALWAYS C94.3 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION | POSSIBLE REQUIREMENT FOR MORPHOLOGY CODE
CorrelationSNOMED CT source code to target map code correlation not specified