Assimakopoulos Stelios F, Michalopoulou Sotiria, Melachrinou Maria, Giannakoulas Nikolaos, Papakonstantinou Christos, Lekkou Alexandra, Gogos Charalambos
Department of Internal Medicine, School of Medicine, University of Patras, Patras, Greece.
Am J Med Sci. 2007 Dec;334(6):493-6. doi: 10.1097/MAJ.0b013e318068b5fd.
Patients with primary Sjögren syndrome frequently present hematologic abnormalities, consisting mainly of immune cytopenias. Pure red cell aplasia is a very rare complication of primary Sjögren syndrome. This is the first report in the literature describing the development of pure red cell aplasia combined with autoimmune hemolytic anemia in a 74-year-old woman with primary Sjögren syndrome. In our patient, despite administration of diverse therapeutic schemes, such as corticosteroids, immunomodulating agents (intravenous immune globulin), immunosuppressive drugs (cyclophosphamide), and novel treatment options (monoclonal antibody directed against the CD20 antigen), no response was achieved. The present case suggests that the possibility of comorbid connective tissue disease should be a diagnostic consideration in patients with acquired pure red cell aplasia and autoimmune hemolytic anemia. Although most of the hematologic abnormalities that occur in primary Sjögren syndrome are not clinically significant, serious and difficult-to-treat hematologic complications may also occur.
原发性干燥综合征患者常出现血液学异常，主要表现为免疫性血细胞减少。纯红细胞再生障碍是原发性干燥综合征一种非常罕见的并发症。本文是文献中首次报道一名74岁原发性干燥综合征女性患者出现纯红细胞再生障碍合并自身免疫性溶血性贫血。在我们的患者中，尽管采用了多种治疗方案，如皮质类固醇、免疫调节剂（静脉注射免疫球蛋白）、免疫抑制药物（环磷酰胺）以及新的治疗选择（抗CD20抗原单克隆抗体），但均未取得疗效。本病例提示，对于获得性纯红细胞再生障碍和自身免疫性溶血性贫血患者，应考虑合并结缔组织病的可能性。虽然原发性干燥综合征中出现的大多数血液学异常在临床上并不显著，但也可能发生严重且难以治疗的血液学并发症。
