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Multifocal lymphangioendotheliomatosis-thrombocytopenia syndrome

Disease definition

A rare lymphatic system anomaly characterized by 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, 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.

ORPHA:464321

Classification level: Disorder
  • Synonym(s):
    • Cutaneovisceral angiomatosis-thrombocytopenia syndrome
    • MLT
    • Multifocal lymphangioendotheliomatosis with thrombocytopenia
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: Neonatal, Infancy
  • ICD-10: D18.1
  • OMIM: -
  • UMLS: -
  • MeSH: -
  • GARD: -
  • MedDRA: -
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