本文报道了1例以头痛和复视为首发症状的嗜血细胞综合征（hemophagocytic syndrome,HPS）成人患者的临床诊治过程。该例女性患者55岁,初始症状表现为头痛和复视,继而出现高热;实验室检查结果显示为全血细胞减少、肝功能异常（包括血清谷丙转氨酶、谷草转氨酶和乳酸脱氢酶水平升高）、高三酰甘油血症、低纤维蛋白原血症和高铁蛋白血症;骨髓穿刺活检结果显示骨髓有核细胞增生减低、3系造血细胞减少,可见嗜血细胞吞噬血小板和中性粒细胞,诊断为HPS。该病例报道提示,对于临床上出现头痛、复视和高热以及外周血检查提示全血细胞减少的患者,应高度怀疑继发性HPS的可能,而尽早进行骨髓穿刺活检是明确诊断的关键。
Abstract
This paper reports one adult case presenting with headache and diplopia as initial symptoms of hemophagocytic syndrome (HPS), describing the diagnostic process and treatment outcomes, in order to improve the understanding of HPS complicated by nervous system involvement. A 55-year-old female adult case presented with headache and diplopia as initial symptoms followed by high fever was hospitalized in Department of Neurology, Renji Hospital, Shanghai Jiao Tong University School of Medicine. Admission laboratory tests revealed pancytopenia, abnormal liver function (elevated serum levels of alanine aminotransferase, aspartate aminotransferase, and lactate dehydrogenase), hypertriglyceridemia, hypofibrinogenemia and hyperferritinaemia. The results of bone marrow aspiration and biopsy showed decreased proliferation of nucleated bone marrow cells, reduction of all three hematopoietic cell lines, and hemophagocytosis of neutrophils and platelets, indicating the diagnosis of HPS. This case report suggests that when a patient presenting with headache, diplopia, fever, and reduction of peripheral whole blood cells should be highly suspected for the possibility of secondary HPS, and the early bone marrow aspiration and biopsy is the key to make a definite diagnosis.
关键词
嗜血细胞综合征 /
神经系统 /
头痛 /
复视 /
成年人
Key words
Hemophagocytic syndrome /
Nervous system /
Headache /
Diplopia /
Adults
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