Department of Pediatric Hematology, Karadeniz Technical University, Trabzon-Turkey.
J Microbiol Immunol Infect. 2010 Apr;43(2):159-62. doi: 10.1016/S1684-1182(10)60025-4.
Hemophagocytic syndrome (HS) may be primary, or secondary, to malignancy, or to metabolic, collagen vascular, and infectious diseases such as brucellosis, miliary tuberculosis and some viral and fungal infections. The diagnostic findings of HS are high fever, hepatosplenomegaly, cytopenia, high serum ferritin and triglycerides, and low serum fibrinogen levels. Brucellosis is a zoonotic disease, with fever, fatigue, sweating, arthritis, hepatosplenomegaly, lymphadenopathy, and cytopenia being the most common symptoms and findings. Hematological manifestations of the disease may include anemia, leucopenia, leukocytosis, thrombocytopenia, and thrombocytosis. Brucellosis may occur in association with HS. Here, we describe brucellosis associated HS in an 8 year-old male patient. The patient was admitted to our clinic with weight loss, arthralgia, prolonged fever, sweating, and fatigue. Physical and laboratory findings revealed hepatosplenomegaly, pancytopenia, elevated serum transaminases, triglycerides, lactate dehydrogenase, and ferritin, and with erythrocytes, leukocytes, and thrombocytes phagocytosed by macrophages indicating hemophagocytosis. The Brucella agglutination test was positive. The patient improved after treatment with Rifampin (15 mg/kg/day) and trimethoprim-sulfamethoxazole (10 mg/kg/day).
噬血细胞综合征（HS）可能是原发性的，也可能是恶性肿瘤、代谢性疾病、胶原血管疾病或感染性疾病的继发性疾病，如布鲁氏菌病、粟粒性肺结核和某些病毒性和真菌性感染。HS 的诊断发现包括高热、肝脾肿大、细胞减少、血清铁蛋白和甘油三酯升高以及血清纤维蛋白原水平降低。布鲁氏菌病是一种人畜共患疾病，最常见的症状和发现包括发热、疲劳、出汗、关节炎、肝脾肿大、淋巴结病和细胞减少。该疾病的血液学表现可能包括贫血、白细胞减少、白细胞增多、血小板减少和血小板增多。布鲁氏菌病可能与 HS 同时发生。在这里，我们描述了一名 8 岁男性患者的布鲁氏菌病相关 HS。该患者因体重减轻、关节炎、长期发热、出汗和疲劳而入院。体格检查和实验室发现显示肝脾肿大、全血细胞减少、血清转氨酶、甘油三酯、乳酸脱氢酶和铁蛋白升高，以及巨噬细胞吞噬红细胞、白细胞和血小板，表明发生噬血细胞现象。布氏菌素凝集试验阳性。患者接受利福平（15mg/kg/天）和甲氧苄啶-磺胺甲恶唑（10mg/kg/天）治疗后好转。
