Shakir Asiya K, Yu Zhongxin, Altaf Muhammad Adnan
Departments of Pediatrics, Division of Pediatric, Gastroenterology, Hepatology, and Nutrition.
Pathology, Section of Pediatric Pathology, University of Oklahoma, Oklahoma City, OK.
J Pediatr Hematol Oncol. 2019 Nov;41(8):e534-e537. doi: 10.1097/MPH.0000000000001324.
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.
多灶性淋巴管内皮瘤病伴血小板减少症是一种罕见疾病，其特征为多发皮肤和胃肠道（GI）血管病变以及对血小板和血细胞输注难治的血小板减少症。在此情况下，胃肠道出血可能危及生命。我们报告一例3个月大男性婴儿的多灶性淋巴管内皮瘤病伴血小板减少症，该患儿仅有胃肠道受累，在3个月大时确诊。诊断后，患者接受了每日氨甲环酸、奥曲肽滴注和皮质类固醇治疗，为期13个月；他在2岁时症状完全缓解，5岁时身高和体重增长正常。该病例补充了文献中关于该疾病表型变异性、长期临床病程和胃肠道出血管理方面的数据匮乏情况。
