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Expanding the phenotype of multifocal lymphangioendotheliomatosis with thrombocytopenia. Pediatr Blood Cancer 2009 Apr;52(4):531-4

Date

12/06/2008

Pubmed ID

19058203

DOI

10.1002/pbc.21766

Scopus ID

2-s2.0-62449109084   24 Citations

Abstract

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 M

Authors

Meghen B. Browning MD Associate Professor in the Pediatrics department at Medical College of Wisconsin
Joseph E. Kerschner MD Provost, Executive Vice President, Dean, Professor in the School of Medicine Administration department at Medical College of Wisconsin
Paula 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

Female
Gastrointestinal Hemorrhage
Gastrointestinal Tract
Humans
Infant
Lung
Male
Phenotype
Skin
Telangiectasia, Hereditary Hemorrhagic
Thrombocytopenia
Vascular Malformations