Palomera Bernal L, García Díez I, Barbudo Merino A, Criado Montilla J, Gea Malpica A
Servicio de Hematología-Hemoterapia, Hospital Comarcal Infanta Margarita, Cabra, Córdoba.
An Med Interna. 1990 Aug;7(8):411-5.
Reactive hemophagocytic syndrome (RHS) or hemophagocytic histiocytosis is a disease with anatomo-pathological features of systemic proliferation of non-neoplastic histiocytes, with prominent hemophagocytosis, associated to infection of other diseases. The cases of three patients afflicted with RHS are presented. 2 of them secondary to a brucellosis and the other of unknown origin. The clinical features were similar: high fever, wasting, and splenomegaly. Pancytopenia existed together with liver disfunction, CID and hyperferremia. Marrow infiltration of reactive histiocytes with important hemophagocytic phenomenon, demonstrated by aspirated and bone marrow biopsies, were observed in all cases. Studies of the immunology system were performed, showing changes in two of them. All of them fully recovered after antibiotic treatment.
反应性噬血细胞综合征(RHS)或噬血细胞性组织细胞增多症是一种具有非肿瘤性组织细胞全身性增殖的解剖病理学特征的疾病,伴有明显的噬血细胞现象,与其他疾病的感染相关。本文报道了3例RHS患者。其中2例继发于布鲁氏菌病,另1例病因不明。他们的临床特征相似:高热、消瘦和脾肿大。全血细胞减少伴肝功能障碍、凝血功能障碍和高铁血症。所有病例均通过骨髓穿刺和活检观察到反应性组织细胞浸润并伴有明显的噬血细胞现象。对免疫系统进行了研究,其中2例出现了变化。所有患者经抗生素治疗后均完全康复。