title: Agranulocytosis and mixed-type autoimmune hemolytic anemia in primary sjögren's syndrome: a case report and review of the literature.
abstract: Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease that presents with sicca symptoms of the main mucosal surfaces. Patients with pSS have a broad spectrum of laboratory features, such as cytopenias and hypergammaglobulinemia. Although hematological abnormalities are usually seen in pSS patients, agranulocytosis and autoimmune hemolytic anemia (AIHA) are rare. Here we describe a 40-year-old woman with pSS who developed both agranulocytosis and mixed-type AIHA. An increased risk of malignancies has also been reported in pSS patients with hematological changes. Although there is no evidence of malignancies, this patient should be closely followed up in case of developing lymphoma.
authors.affiliation: Department of Internal Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
authors.forename: Lin
authors.initials: L
authors.lastname: Qiao
authors.affiliation: Department of Internal Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
authors.forename: Jing
authors.initials: J
authors.lastname: Chen
authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
authors.forename: Xiao-Mei
authors.initials: XM
authors.lastname: Leng
authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
authors.forename: Wen
authors.initials: W
authors.lastname: Zhang
authors.affiliation: Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
authors.forename: Bing
authors.initials: B
authors.lastname: Han
authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
authors.forename: Yan
authors.initials: Y
authors.lastname: Zhao
authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
authors.forename: Xiao-Feng
authors.initials: XF
authors.lastname: Zeng
mesh_terms: D061605:Administration, Intravenous; D000284:Administration, Oral; D000328:Adult; D000380:Agranulocytosis / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D000744:Anemia, Hemolytic, Autoimmune / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D057915:Drug Substitution; D004359:Drug Therapy, Combination; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D007958:Leukocyte Count; D010976:Platelet Count; D020551:Pulse Therapy, Drug; D012859:Sjogren's Syndrome / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology*; D016896:Treatment Outcome
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents
keywords: Sjögren's syndrome; agranulocytosis; autoimmune hemolytic anemia