Eakle J F, Bressoud P F
Department of Veterans Affairs Medical Center (VAMC), Louisville, KY, USA.
J Ky Med Assoc. 2000 Apr;98(4):161-5.
Hemophagocytosis is an uncommon disorder characterized by proliferation of histiocytes that actively engulf other hematopoietic cells causing cytopenia. Reactive or secondary hemophagocytosis is very rare in healthy adults in the US. Various infectious, as well as neoplastic and immunologic etiologies of reactive hemophagocytosis have been reported. It is a non-malignant, reactive disorder characterized by hemophagocytosis in the bone marrow and reticuloendothelial system (RES) resulting in pancytopenia, fever, hepatic dysfunction, and disseminated intravascular coagulation (DIC). No consensus exists in the literature regarding optimal treatment of virus-associated hemophagocytic syndrome (VAHS). We report a case of VAHS in a previously healthy immunocompetent male and review the diagnosis and management of this rare disorder.
噬血细胞作用是一种罕见的疾病，其特征是组织细胞增殖，这些组织细胞会主动吞噬其他造血细胞，导致血细胞减少。在美国，反应性或继发性噬血细胞作用在健康成年人中非常罕见。已经报道了反应性噬血细胞作用的各种感染性、肿瘤性和免疫性病因。它是一种非恶性的反应性疾病，其特征是骨髓和网状内皮系统（RES）中的噬血细胞作用，导致全血细胞减少、发热、肝功能障碍和弥散性血管内凝血（DIC）。关于病毒相关性噬血细胞综合征（VAHS）的最佳治疗方法，文献中尚无共识。我们报告了一例先前健康的免疫功能正常男性患VAHS的病例，并回顾了这种罕见疾病的诊断和管理。
