Virus‐associated hemophagocytic syndrome A benign histiocytic proliferation distinct from malignant histiocytosis
Virus‐associated hemophagocytic syndrome A benign histiocytic proliferation distinct from malignant histiocytosis
复制标题
病毒相关噬血细胞综合征 一种不同于恶性组织细胞增多症的良性组织细胞增殖
作者：
R. Risdall;R. Mckenna;M. Nesbit;W. Krivit;H. Balfour;R. Simmons;R. Brunning
19例骨髓涂片显示组织细胞增生伴明显噬血症的患者，发现有与活动性病毒感染相关的临床病理综合征。高热、体质症状、肝功能、凝血异常和外周血细胞减少是特征性表现。常出现肝脾肿大、淋巴结病变、双侧肺浸润和皮疹。14例患者免疫抑制。14例患者被疱疹群病毒感染，1例被腺病毒感染。大多数患者的骨髓也显示出粒细胞和红细胞生成减少，巨核细胞数量正常到增加。治疗一般包括支持治疗和停用免疫抑制药物。13名患者康复。淋巴结活检和尸检资料显示全身性组织细胞增生伴噬血细胞增多。本文讨论了该疾病与家族性噬血细胞网状病、家族性红细胞性淋巴组织细胞病、组织细胞性髓样网状病和恶性组织细胞病的关系。免疫抑制和细胞毒性治疗可能是治疗这种病毒相关综合征的禁忌。
Nineteen patients whose bone marrow smears showed histiocytic hyperplasia with prominent hemophagocytosis were found to have a clinicopathologic syndrome associated with active viral infection. High fever, constitutional symptoms, liver function, and coagulation abnormalities and peripheral blood cytopenias were characteristic findings. Hepatosplenomegaly, lymphadenopathy, bilateral pulmonary infiltrates, and skin rash were often present. Fourteen of the patients were immunosuppressed. Active infection by herpes group viruses was documented in 14 patients and by adenovirus in 1. The bone marrow of most patients also showed decreased granulopoiesis and erythropoiesis with normal to increased numbers of megakaryocytes. Treatment generally consisted of supportive therapy and withdrawal of immunosuppressive drugs. Thirteen patients recovered. Lymph node biopsy and autopsy material showed generalized histiocytic hyperplasia with hemophagocytosis. The relationship of this disorder to familial hemophagocytic reticulosis, familial erythrophagocytic lymphohistiocytosis, histiocytic medullary reticulosis, and malignant histiocytosis is discussed. Immunosuppressive and cytotoxic therapy may be contraindicated in the treatment of this virus‐associated syndrome.