Perrino Carmen M, Dehner Louis P, Hartman Mary E, Agarwal Ashima
1 Department of Pathology and Immunology, Washington University, Campus Box 8118, 660 South Euclid Ave., St. Louis, MO 63110, USA.
Pediatr Dev Pathol. 2014 Jan-Feb;17(1):44-9. doi: 10.2350/13-05-1339-CR.1. Epub 2013 Oct 7.
Myelodysplastic syndrome (MDS) and pulmonary tumor thrombotic microangiopathy (PTTM) are independently rare in the pediatric population. This report describes an 11-year-old male patient who initially presented with respiratory distress and cardiovascular collapse. A large left main pulmonary artery embolus and multiple, smaller pulmonary thromboemboli were widely dispersed throughout both lungs. Despite aggressive supportive care, he died within seven hours of admission. A complete postmortem examination was performed, leading to the diagnoses of primary MDS and microthrombi in the lungs, including the characteristic fibroproliferative lesions seen in PTTM. Individually, both conditions are extremely uncommon, and therefore the coincidence of these 2 conditions in a child is singularly unique.
骨髓增生异常综合征（MDS）和肺肿瘤血栓性微血管病（PTTM）在儿科人群中各自都很罕见。本报告描述了一名11岁男性患者，最初表现为呼吸窘迫和心血管衰竭。一个大的左主肺动脉栓子和多个较小的肺血栓栓子广泛散布于双肺。尽管给予了积极的支持治疗，但他在入院后7小时内死亡。进行了完整的尸检，诊断为原发性MDS和肺部微血栓形成，包括PTTM中所见的特征性纤维增生性病变。单独来看，这两种情况都极为罕见，因此这两种情况在一名儿童中同时出现是独一无二的。
