Expanding the phenotype of multifocal lymphangioendotheliomatosis with thrombocytopenia. Pediatr Blood Cancer 2009 Apr;52(4):531-4
Date
12/06/2008Pubmed ID
19058203DOI
10.1002/pbc.21766Scopus ID
2-s2.0-62449109084 (requires institutional sign-in at Scopus site) 28 CitationsAbstract
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.
Author List
Maronn M, Catrine K, North P, Browning MB, Kerschner JE, Noel R, Drolet BA, Kelly MAuthors
Joseph E. Kerschner MD Provost, Executive Vice President, Dean, Professor in the School of Medicine Administration department at Medical College of WisconsinPaula E. North MD, PhD Professor in the Pathology department at Medical College of Wisconsin
MESH terms used to index this publication - Major topics in bold
FemaleGastrointestinal Hemorrhage
Gastrointestinal Tract
Humans
Infant
Lung
Male
Phenotype
Skin
Telangiectasia, Hereditary Hemorrhagic
Thrombocytopenia
Vascular Malformations