Long-term Complications and Management of Gastrointestinal Bleeding in Multifocal Lymphangioendotheliomatosis
| Autor: | Zhongxin Yu, Muhammad A. Altaf, Asiya K. Shakir |
|---|---|
| Rok vydání: | 2019 |
| Předmět: |
Male
medicine.medical_specialty Gastrointestinal bleeding Octreotide Disease Gastroenterology 03 medical and health sciences 0302 clinical medicine Refractory Adrenal Cortex Hormones Internal medicine medicine Humans Platelet Multifocal lymphangioendotheliomatosis Lymphangioma business.industry Infant Hematology medicine.disease Thrombocytopenia Oncology 030220 oncology & carcinogenesis Aminocaproic Acid Pediatrics Perinatology and Child Health Aminocaproic acid Gastrointestinal Hemorrhage business 030215 immunology medicine.drug Rare disease |
| Zdroj: | Journal of Pediatric Hematology/Oncology. 41:e534-e537 |
| ISSN: | 1077-4114 |
| DOI: | 10.1097/mph.0000000000001324 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
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