Congenital thrombocytopenia
Finding of lymph node and lymphatics
Lymphatic malformation
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Multifocal lymphangioendotheliomatosis, thrombocytopenia syndrome (disorder)
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 interpretationAbnormal
InterpretsHemostatic function
Has interpretationBelow reference range
InterpretsPlatelet count
Associated morphologyCongenital lymphatic malformation
Finding siteStructure of lymphatic vessel
OccurrenceCongenital
Pathological processPathological developmental process
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