Takenaka H, Kishimoto S, Ichikawa R, Shibagaki R, Kubota Y, Yamagata N, Gotoh H, Fujita N, Yasuno H
Department of Dermatology, Kyoto Prefectural University of Medicine, Japan.
Br J Dermatol. 1998 Nov;139(5):877-80. doi: 10.1046/j.1365-2133.1998.02517.x.
Haemophagocytic syndrome is a systemic clinicopathological entity characterized by systemic proliferation of benign haemophagocytic histiocytes, fever, cytopenia, abnormal liver function and, frequently, coagulopathy and hepatosplenomegaly. Its occurrence has been documented in association with viral, bacterial, fungal and parasitic infections, a wide spectrum of malignant neoplasms, autoimmune diseases and drugs. We report a case of rubella virus-associated haemophagocytic syndrome in a previously healthy 29-year-old woman. Blood tests showed cytopenia, especially severe thrombocytopenia, liver dysfunction, hyperferritinaemia and hypercytokinaemia. Bone marrow examination showed many mature histiocytes with active haemophagocytosis. A skin biopsy from the rash revealed perivascular lymphohistiocytic infiltrates with haemophagocytic histiocytes in the upper and mid-dermis. The patient was treated with antibiotics and immunoglobulin, and by supportive measures including platelet transfusion, and recovered completely.
噬血细胞综合征是一种系统性临床病理实体,其特征为良性噬血细胞组织细胞的系统性增殖、发热、血细胞减少、肝功能异常,且常伴有凝血病和肝脾肿大。已有文献记载其与病毒、细菌、真菌和寄生虫感染、多种恶性肿瘤、自身免疫性疾病及药物有关。我们报告一例先前健康的29岁女性患风疹病毒相关噬血细胞综合征的病例。血液检查显示血细胞减少,尤其是严重血小板减少、肝功能异常、高铁蛋白血症和高细胞因子血症。骨髓检查显示许多成熟组织细胞伴有活跃的噬血现象。皮疹部位的皮肤活检显示真皮上部和中部有血管周围淋巴细胞组织细胞浸润,并伴有噬血细胞组织细胞。该患者接受了抗生素和免疫球蛋白治疗,并采取了包括血小板输注在内的支持措施,最终完全康复。