Nagao Yoshiro, Yamanaka Hiromi, Harada Hiromasa
Department of Internal Medicine, Yao Tokushukai General Hospital, 1-11 Wakakusa-cho, Yao city, Osaka, 581-0011, Japan.
J Med Case Rep. 2012 Feb 14;6:63. doi: 10.1186/1752-1947-6-63.
Hypereosinophilic syndrome is defined as a prolonged state (more than six months) of eosinophilia (greater than 1500 cells/μL), without an apparent etiology and with end-organ damage. Hypereosinophilic syndrome can cause coagulation abnormalities. Among hypereosinophilic syndrome types, the lymphocytic variant (lymphocytic hypereosinophilic syndrome) is derived from a monoclonal proliferation of T lymphocytes. Here, we describe the case of a patient with lymphocytic hypereosinophilic syndrome who presented with a coagulation abnormality. To the best of our knowledge, this is the first such report including a detailed clinical picture and temporal cytokine profile.
A 77-year-old Japanese man presented to our facility with massive hematuria and hypereosinophilia (greater than 2600 cells/μl). His eosinophilia first appeared five years earlier when he developed femoral artery occlusion. He manifested with multiple hematomas and prolonged activated partial thromboplastin time. His IgG4 level was remarkably elevated (greater than 2000 mg/dL). Polymerase chain reaction tests of peripheral blood and bone marrow identified lymphocytic hypereosinophilic syndrome. His prolonged activated partial thromboplastin time was found to be due to acquired hemophilia. Glucocorticoids suppressed both the hypereosinophilia and coagulation abnormality. However, tapering of glucocorticoids led to a relapse of the coagulation abnormality alone, without eosinophilia. Tumor necrosis factor α, interleukin-5, and/or eotaxin-3 may have caused the hypereosinophilia, and interleukin-10 was correlated with the coagulation abnormality.
To the best of our knowledge, this is the first case in which lymphocytic hypereosinophilic syndrome and IgG4-related disease have overlapped. In addition, our patient is only the second case of hypereosinophilic disease that manifested with acquired hemophilia. Our patient relapsed with the coagulation abnormality alone, without eosinophilia. This report shows that the link between eosinophilia, IgG4, and clinical manifestations is not simple and provides useful insight into the immunopathology of hypereosinophilic syndrome and IgG4-related disease.
高嗜酸性粒细胞综合征被定义为嗜酸性粒细胞增多（大于1500个细胞/μL）的持续状态（超过六个月），无明显病因且伴有终末器官损害。高嗜酸性粒细胞综合征可导致凝血异常。在高嗜酸性粒细胞综合征类型中，淋巴细胞变异型（淋巴细胞性高嗜酸性粒细胞综合征）源自T淋巴细胞的单克隆增殖。在此，我们描述了一例患有淋巴细胞性高嗜酸性粒细胞综合征且伴有凝血异常的患者。据我们所知，这是首例包含详细临床情况和时间性细胞因子谱的此类报告。
一名77岁的日本男性因大量血尿和高嗜酸性粒细胞增多（大于2600个细胞/μl）前来我院就诊。他的嗜酸性粒细胞增多首次出现在五年前，当时他出现了股动脉闭塞。他表现为多处血肿和活化部分凝血活酶时间延长。他的IgG4水平显著升高（大于2000mg/dL）。外周血和骨髓的聚合酶链反应检测确诊为淋巴细胞性高嗜酸性粒细胞综合征。发现他活化部分凝血活酶时间延长是由于获得性血友病。糖皮质激素抑制了高嗜酸性粒细胞增多和凝血异常。然而，糖皮质激素减量导致仅凝血异常复发，而无嗜酸性粒细胞增多。肿瘤坏死因子α、白细胞介素-5和/或嗜酸性粒细胞趋化因子-3可能导致了高嗜酸性粒细胞增多，白细胞介素-10与凝血异常相关。
据我们所知，这是首例淋巴细胞性高嗜酸性粒细胞综合征与IgG4相关疾病重叠的病例。此外，我们的患者是第二例表现为获得性血友病的高嗜酸性粒细胞疾病患者。我们的患者仅凝血异常复发，无嗜酸性粒细胞增多。本报告表明嗜酸性粒细胞增多、IgG4与临床表现之间的联系并不简单，并为高嗜酸性粒细胞综合征和IgG4相关疾病的免疫病理学提供了有用的见解。
