title: [Exacerbation of autoimmune neutropenia to agranulocytosis in association with severe autoimmune thrombocytopenia and hemolytic anemia in a patient with Sjögren's syndrome].
abstract: A 73-year-old woman with Sjögren's syndrome and autoimmune neutropenia (AIN) associated with large granular lymphocytosis of the polyclonal T cell type, demonstrated autoimmune thrombocytopenia (AIT) at diagnosis of sigmoid colon cancer. Ten months later, both AIN and AIT had exacerbated to agranulocytosis and severe thrombocytopenia below 10×10(9)/L, respectively. There were no dysplastic features of bone marrow hematopoietic cells. Furthermore, an in vitro assay of hematopoietic progenitors showed normal granuloid and erythroid colony formation. Although we serially treated her with prednisolone (oral), filgrastim, intravenous high-dose immunoglobulin infusion, cyclophosphamide (oral), danazol, cyclosporine A (oral), and rituximab, number of neutrophils and platelets elevated only temporarily. During the course of agranulocytosis and severe thrombocytopenia, the patient also developed autoimmune hemolytic anemia (AIHA). She died of pneumonia 5 months after the onset of agranulocytosis. This case is very unique and novel in terms of autoimmune phenomena simultaneously directed to granulocytes, platelets, and red blood cells under the background of Sjögren's syndrome.
authors.affiliation: Departments of Hematology and Clinical Immunology, Kobe City Medical Center, General Hospital.
authors.forename: Sonoko
authors.initials: S
authors.lastname: Shimoji
authors.forename: Yohko
authors.initials: Y
authors.lastname: Takiuchi
authors.forename: Hayato
authors.initials: H
authors.lastname: Maruoka
authors.forename: Daichi
authors.initials: D
authors.lastname: Inoue
authors.forename: Takaharu
authors.initials: T
authors.lastname: Kimura
authors.forename: Minako
authors.initials: M
authors.lastname: Mori
authors.forename: Yuya
authors.initials: Y
authors.lastname: Nagai
authors.forename: Katuhiro
authors.initials: K
authors.lastname: Togami
authors.forename: Sumie
authors.initials: S
authors.lastname: Tabata
authors.forename: Masayuki
authors.initials: M
authors.lastname: Kurata
authors.forename: Akiko
authors.initials: A
authors.lastname: Matsushita
authors.forename: Kenichi
authors.initials: K
authors.lastname: Nagai
authors.forename: Takayuki
authors.initials: T
authors.lastname: Takahashi
mesh_terms: D000368:Aged; D000380:Agranulocytosis / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D000744:Anemia, Hemolytic, Autoimmune / Q000209:etiology* / Q000276:immunology; D001327:Autoimmune Diseases / Q000209:etiology* / Q000276:immunology; D015551:Autoimmunity; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D009503:Neutropenia / Q000209:etiology* / Q000276:immunology; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D012720:Severity of Illness Index; D012859:Sjogren's Syndrome / Q000150:complications* / Q000276:immunology