Expanding the phenotype of multifocal lymphangioendotheliomatosis with thrombocytopenia |
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| Authors: | Mandi Maronn MD Kristina Catrine MD Paula North MD PhD Meghen B Browning MD Joseph E Kerschner MD FACS FAAP Richard Noel MD PhD Beth A Drolet MD Michael Kelly MD PhD |
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| Institution: | 1. Department of Dermatology, Medical College of Wisconsin, Milwaukee, Wisconsin;2. Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin;3. Department of Pathology, Children's Hospital of Wisconsin, Milwaukee, Wisconsin;4. Medical Director.;5. Department of Otolaryngology, Children's Hospital of Wisconsin, Milwaukee, Wisconsin;6. Department of Gastroenterology, Children's Hospital of Wisconsin, Milwaukee, Wisconsin;7. Department of Dermatology, Children's Hospital of Wisconsin, Milwaukee, Wisconsin;8. Department of Oncology, Children's Hospital of Wisconsin, Milwaukee, Wisconsin |
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| 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. Pediatr Blood Cancer 2009;52:531–534. © 2008 Wiley‐Liss, Inc. |
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| Keywords: | gastrointestinal bleeding thalidomide thrombocytopenia vascular malformation |
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