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multifocale lymfangio-endotheliomatose met trombocytopenie (aandoening)
multifocale lymfangio-endotheliomatose met trombocytopenie
MLT
Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome
Cutaneovisceral angiomatosis, thrombocytopenia syndrome
MLT - multifocal lymphangioendotheliomatosis with thrombocytopenia
A rare lymphatic system anomaly with characteristics of multifocal congenital and progressive vascular lesions of the skin, gastrointestinal tract and occasionally other anatomic sites, causing potentially life-threatening thrombocytopenic coagulopathy. Macroscopically the lesions appear as round to oval red-brown plaques and as large as a few centimeters in diameter. Histopathologically they consist of dilated thin-walled vessels with variable endothelial hyperplasia, positive for lymphatic endothelial cell markers and resembling benign lymphangioendothelioma.
Id1208725005
StatusPrimitive
Has interpretationafwijkend
Interpretshemostase
Has interpretationonder referentiebereik
InterpretsPlatelet count
ICD-10 complex map reference set
TargetD18.1
RuleTRUE
AdviceALWAYS D18.1 | 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