Dalle J H, Dollfus C, Leverger G, Landman-Parker J, Tabone M D, Adam M, Courpotin C, Lasfargues G
Département de pédiatrie Edmond-Lesné, hôpital d'enfants Armand-Trousseau, Paris, France.
Arch Pediatr. 1995 May;2(5):442-6. doi: 10.1016/0929-693x(96)81179-1.
The hemophagocytic syndrome has previously been reported in different infectious diseases (EBV, CMV, tuberculosis...) but rarely in adults with AIDS and never in children suffering from AIDS.
A hemophagocytic syndrome was recognized during the follow-up of 3 children with AIDS. The first, a 9-year-old girl developed an acute EBV coinfection and was treated with shots of corticosteroids and vepesid but died shortly afterwards. The second patient, a 3-year-old girl was infected with Aspergillus fumigatus for which she was given amphotericin B with a rapid improvement. The third patient, an 8-year-old boy had multi-resistant Streptococcus pneumoniae otitis and pneumonitis; his condition improved rapidly with adapted antibiotherapy.
The HIV-hemophagocytic syndrome is not exceptional in HIV infection because of the association of immunodeficiency and resulting superinfections. Its diagnosis and treatment should be etiologic. Severe cases without etiology could benefit from chemotherapy.
Management and outcome of this potentially lethal syndrome might depend on the identification of a curable infectious cause.
噬血细胞综合征此前已在不同的传染病（如EB病毒、巨细胞病毒、结核病等）中报道过，但在成年艾滋病患者中很少见，在儿童艾滋病患者中从未有过报道。
在对3名艾滋病儿童的随访过程中发现了噬血细胞综合征。第一名是一名9岁女孩，并发急性EB病毒感染，接受了皮质类固醇和依托泊苷注射治疗，但不久后死亡。第二名患者是一名3岁女孩，感染了烟曲霉，接受两性霉素B治疗后病情迅速好转。第三名患者是一名8岁男孩，患有多重耐药性肺炎链球菌中耳炎和肺炎；经适当的抗生素治疗后病情迅速改善。
由于免疫缺陷及由此导致的重叠感染，HIV相关性噬血细胞综合征在HIV感染中并不罕见。其诊断和治疗应针对病因。病因不明的严重病例可能受益于化疗。
这种潜在致命综合征的管理和预后可能取决于可治愈感染病因的识别。
