Expanding the phenotype of multifocal lymphangioendotheliomatosis with thrombocytopenia.

Published

Journal Article

Multifocal lymphangioendotheliomatosis with thrombocytopenia is characterized by vascular skin and gastrointestinal (GI) tract lesions, thrombocytopenia, and GI bleeding. The first patient had scattered red macules and subcutaneous nodules on the skin with involvement of the lungs, liver, omentum, and right kidney. At 10 months of age he continues to have severe GI bleeding. The second patient had innumerable vascular plaques on the skin plus muscle, bone, lung, liver, and brain involvement. She died from respiratory failure at 8 months of age due to brainstem involvement. Both patients required aggressive management of GI bleeding, but had quite different skin findings and long-term outcomes.

Full Text

Duke Authors

Cited Authors

  • Maronn, M; Catrine, K; North, P; Browning, MB; Kerschner, JE; Noel, R; Drolet, BA; Kelly, M

Published Date

  • April 2009

Published In

Volume / Issue

  • 52 / 4

Start / End Page

  • 531 - 534

PubMed ID

  • 19058203

Pubmed Central ID

  • 19058203

Electronic International Standard Serial Number (EISSN)

  • 1545-5017

International Standard Serial Number (ISSN)

  • 1545-5009

Digital Object Identifier (DOI)

  • 10.1002/pbc.21766

Language

  • eng