| 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. |
| | Id | 1208725005 | | Status | Primitive |
| SNOMED CT to Orphanet simple map | 464321 |
| SNOMED CT to ICD-10 extended map | | Target | D18.1 | | Rule | TRUE | | Advice | ALWAYS D18.1 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION | POSSIBLE REQUIREMENT FOR MORPHOLOGY CODE | | Correlation | SNOMED CT source code to target map code correlation not specified |
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