Keller F G, Kurtzberg J
Department of Pediatrics, West Virginia University School of Medicine, Morgantown 26506.
Am J Pediatr Hematol Oncol. 1994 Nov;16(4):368-71.
We report a case of infection-associated hemophagocytic syndrome in the setting of disseminated histoplasmosis.
A 6-year-old boy with chronic mucocutaneous candidiasis developed a fulminant hemophagocytic syndrome. Evaluation for an infectious cause included bacterial, mycobacterial, viral, and fungal cultures, serological assessment, and histological examination of bone marrow and bronchoalveolar lavage fluid.
Culture of bone marrow aspirate, blood, and bronchoalveolar lavage showed disseminated histoplasmosis as the cause for this patient's hemophagocytic syndrome. The patient was treated with amphotericin B with complete resolution of his hemophagocytic syndrome.
Disseminated histoplasmosis is another cause of IAHS in children with an underlying immunodeficiency. Diagnostic difficulties associated with the hemophagocytic syndromes of childhood are discussed.
我们报告一例播散性组织胞浆菌病合并感染相关噬血细胞综合征的病例。
一名患有慢性黏膜皮肤念珠菌病的6岁男孩发生暴发性噬血细胞综合征。对感染病因的评估包括细菌、分枝杆菌、病毒和真菌培养、血清学评估以及骨髓和支气管肺泡灌洗的组织学检查。
骨髓穿刺液、血液和支气管肺泡灌洗的培养显示播散性组织胞浆菌病是该患者噬血细胞综合征的病因。患者接受两性霉素B治疗，噬血细胞综合征完全缓解。
播散性组织胞浆菌病是潜在免疫缺陷儿童发生感染相关噬血细胞综合征的另一病因。文中讨论了儿童噬血细胞综合征相关的诊断困难。
