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Agranulocytosis and mixed‐type autoimmune hemolytic anemia in primary sjögren's syndrome: a case report and review of the literature
Authors:Lin Qiao  Jing Chen  Xiao‐mei Leng  Wen Zhang  Bing Han  Yan Zhao  Xiao‐feng Zeng
Affiliation:1. Department of Internal Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China;2. Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China;3. Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
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.
Keywords:agranulocytosis  autoimmune hemolytic anemia  Sjö  gren's syndrome
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