Shimoji Sonoko, Takiuchi Yohko, Maruoka Hayato, Inoue Daichi, Kimura Takaharu, Mori Minako, Nagai Yuya, Togami Katuhiro, Tabata Sumie, Kurata Masayuki, Matsushita Akiko, Nagai Kenichi, Takahashi Takayuki
Departments of Hematology and Clinical Immunology, Kobe City Medical Center, General Hospital.
Rinsho Ketsueki. 2011 Jul;52(7):535-9.
A 73-year-old woman with Sjögren's syndrome and autoimmune neutropenia (AIN) associated with large granular lymphocytosis of the polyclonal T cell type, demonstrated autoimmune thrombocytopenia (AIT) at diagnosis of sigmoid colon cancer. Ten months later, both AIN and AIT had exacerbated to agranulocytosis and severe thrombocytopenia below 10×10(9)/L, respectively. There were no dysplastic features of bone marrow hematopoietic cells. Furthermore, an in vitro assay of hematopoietic progenitors showed normal granuloid and erythroid colony formation. Although we serially treated her with prednisolone (oral), filgrastim, intravenous high-dose immunoglobulin infusion, cyclophosphamide (oral), danazol, cyclosporine A (oral), and rituximab, number of neutrophils and platelets elevated only temporarily. During the course of agranulocytosis and severe thrombocytopenia, the patient also developed autoimmune hemolytic anemia (AIHA). She died of pneumonia 5 months after the onset of agranulocytosis. This case is very unique and novel in terms of autoimmune phenomena simultaneously directed to granulocytes, platelets, and red blood cells under the background of Sjögren's syndrome.
一名73岁女性，患有干燥综合征及与多克隆T细胞型大颗粒淋巴细胞相关的自身免疫性中性粒细胞减少症（AIN），在乙状结肠癌诊断时出现了自身免疫性血小板减少症（AIT）。10个月后，AIN和AIT分别加重为粒细胞缺乏症和严重血小板减少症，血小板计数低于10×10⁹/L。骨髓造血细胞无发育异常特征。此外，造血祖细胞的体外试验显示粒细胞和红细胞集落形成正常。尽管我们先后用泼尼松龙（口服）、非格司亭、静脉注射大剂量免疫球蛋白、环磷酰胺（口服）、达那唑、环孢素A（口服）和利妥昔单抗对她进行治疗，但中性粒细胞和血小板数量仅暂时升高。在粒细胞缺乏症和严重血小板减少症病程中，患者还出现了自身免疫性溶血性贫血（AIHA）。粒细胞缺乏症发病5个月后，患者死于肺炎。就干燥综合征背景下同时针对粒细胞、血小板和红细胞的自身免疫现象而言，该病例非常独特且新颖。
