title: Primary Sjögren syndrome complicated by autoimmune hemolytic anemia and pure red cell aplasia.
abstract: 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.
authors: Department of Internal Medicine, School of Medicine, University of Patras, Patras, Greece. sassim@upatras.gr
authors: Stelios F
authors: SF
authors: Assimakopoulos
authors: Sotiria
authors: S
authors: Michalopoulou
authors: Maria
authors: M
authors: Melachrinou
authors: Nikolaos
authors: N
authors: Giannakoulas
authors: Christos
authors: C
authors: Papakonstantinou
authors: Alexandra
authors: A
authors: Lekkou
authors: Charalambos
authors: C
authors: Gogos
mesh_terms: D000368:Aged; D000744:Anemia, Hemolytic, Autoimmune / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D001853:Bone Marrow / Q000473:pathology; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D012010:Red-Cell Aplasia, Pure / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D012859:Sjogren's Syndrome / Q000097:blood / Q000150:complications* / Q000188:drug therapy