Komaru Yohei, Higuchi Takakazu, Koyamada Ryosuke, Haji Youichiro, Okada Masato, Kamesaki Toyomi, Okada Sadamu
Division of Hematology, St. Luke's International Hospital, Japan.
Intern Med. 2013;52(20):2343-6. doi: 10.2169/internalmedicine.52.0695.
A 36-year-old woman presented with hemolytic anemia without a reticulocyte response 38 days after delivery. A marked reduction in erythroid cells and an increase in macrophages with active hemophagocytosis were noted in the bone marrow. While conventional Coombs' tests were negative, the level of red blood cell (RBC)-bound immunoglobulin G (IgG) was increased. The patient was diagnosed with primary Sjögren syndrome (pSS) based on her symptoms, positive anti-SS-A antibodies, Coombs-negative autoimmune hemolytic anemia and pure red cell aplasia associated with RBC-bound IgG and hemophagocytosis. The unique presentation was considered to be a consequence of immunological derangement associated with pSS, pregnancy and delivery.
一名36岁女性在分娩38天后出现无网织红细胞反应的溶血性贫血。骨髓中可见红系细胞显著减少，巨噬细胞增多并伴有活跃的噬血细胞现象。虽然传统的库姆斯试验为阴性，但红细胞（RBC）结合的免疫球蛋白G（IgG）水平升高。根据患者的症状、抗SS - A抗体阳性、库姆斯阴性的自身免疫性溶血性贫血以及与RBC结合的IgG和噬血细胞现象相关的纯红细胞再生障碍，该患者被诊断为原发性干燥综合征（pSS）。这种独特的表现被认为是与pSS、妊娠和分娩相关的免疫紊乱的结果。
