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Long-term Complications and Management of Gastrointestinal Bleeding in Multifocal Lymphangioendotheliomatosis

Shakir, Asiya K. MD*; Yu, Zhongxin MD; Altaf, Muhammad Adnan MD*

Journal of Pediatric Hematology/Oncology: October 16, 2018 - Volume Publish Ahead of Print - Issue - p
doi: 10.1097/MPH.0000000000001324
Clinical and Laboratory Observations: PDF Only

Multifocal lymphangioendotheliomatosis with thrombocytopenia is a rare disease characterized by multiple cutaneous and gastrointestinal (GI) vascular lesions and thrombocytopenia refractory to platelet and blood cell transfusions. GI bleeding can become life-threatening in this condition. We report a case of multifocal lymphangioendotheliomatosis with thrombocytopenia in a male infant with isolated GI involvement, diagnosed when he was 3 months old. The patient was managed with daily aminocaproic acid, octreotide drip, and corticosteroids for 13 months after diagnosis; he had complete resolution of symptoms by 2 years of age and showed adequate height and gain by 5 years of age. This case adds to the paucity of data in the literature pertaining to the disease’s phenotypic variability, long-term clinical course, and management of GI bleeding.

Departments of *Pediatrics, Division of Pediatric, Gastroenterology, Hepatology, and Nutrition

Pathology, Section of Pediatric Pathology, University of Oklahoma, Oklahoma City, OK

The authors declare no conflict of interest.

Reprints: Asiya K. Shakir, MD, 1200 Children’s Avenue, Suite 9E, Oklahoma City, OK 73104 (e-mail:

Received March 20, 2018

Accepted August 26, 2018

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