肺泡蛋白沉积症合并先天性红细胞生成异常性贫血：一例报告

Pulmonary alveolar proteinosis in association with congenital dyserythropoietic anemia: a case report.

作者信息

Carden Marcus A, Barman Ashish, Massey Gita

机构信息

Departments of Internal Medicine and Pediatrics, Virginia Commonwealth University, 1001 East Marshall Street, Richmond, VA 23298-0646, USA.

出版信息

Case Rep Pediatr. 2012;2012:624740. doi: 10.1155/2012/624740. Epub 2012 Jun 28.

DOI:10.1155/2012/624740

PMID:22953144

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3395170/

Abstract

A two-year-old girl with congenital dyserythropoietic anemia (CDA) acutely developed fever, tachypnea, and increased oxygen requirement. Chest X-ray revealed bilateral interstitial infiltrates and mild cardiomegaly. Blood cultures grew no infectious agents, while pulmonary specimens grew cytomegalovirus (CMV). Treatment with intravenous ganciclovir was initiated but without response. Final cytologic preparations of bronchoalveolar lavage (BAL) fluid revealed eosinophilic amorphous material consistent with pulmonary alveolar proteinosis (PAP). CDA and PAP are extremely rare disorders in pediatrics. PAP should be considered in patients with hematological disorders who present with acute interstitial pneumonia, after infectious causes are ruled out.

摘要

一名患有先天性红细胞生成异常性贫血（CDA）的两岁女童突然出现发热、呼吸急促和需氧量增加。胸部X线显示双侧间质性浸润和轻度心脏扩大。血培养未发现感染病原体，而肺部标本培养出巨细胞病毒（CMV）。开始使用静脉注射更昔洛韦治疗，但无反应。支气管肺泡灌洗（BAL）液的最终细胞学标本显示嗜酸性无定形物质，符合肺泡蛋白沉积症（PAP）。CDA和PAP在儿科极为罕见。在排除感染原因后，对于患有血液系统疾病且出现急性间质性肺炎的患者，应考虑PAP。