title: Mycophenolate mofetil in primary Sjögren's syndrome: a treatment option for agranulocytosis.
abstract: The Sjögren's syndrome (SS) is an autoimmune disease characterized by a lymphocytic infiltration of salivary and lacrimal glands. Hematological manifestations of primary SS (pSS) usually consist of mild anemia, thrombocytopenia, moderate neutropenia, and lymphopenia. Agranulocytosis is rarely reported and usually responds to immunosuppression. We report the case of a pSS patient who presented with refractory agranulocytosis. Bone marrow biopsy disclosed a hypocellular bone marrow with normal maturation of the granulocytic series. The patient was successively treated with high-dose prednisone, granulocyte-macrophage colony stimulation factor, and cyclosporine, with no hematological response. Mycophenolate mofetil (MMF) was initiated and after two months there was a rise on the white blood cell count. After one year of follow-up, she had neither further neutropenia episodes, nor infectious complications. We conclude that, in pSS refractory agranulocytosis, MMF can be an effective and well-tolerated treatment option.
authors.affiliation: Serviços de Reumatologia e Clínica Médica, Universidade Federal de Santa Catarina. fialhoson@gmail.com
authors.forename: Sonia Cristina de Magalhães Souza
authors.initials: SC
authors.lastname: Fialho
authors.forename: Samuel
authors.initials: S
authors.lastname: Bergamaschi
authors.forename: Fabrício Souza
authors.initials: FS
authors.lastname: Neves
authors.forename: Adriana Fontes
authors.initials: AF
authors.lastname: Zimmermann
authors.forename: Gláucio Ricardo Werner de
authors.initials: GR
authors.lastname: Castro
authors.forename: Ivânio Alves
authors.initials: IA
authors.lastname: Pereira
mesh_terms: D000368:Aged; D000380:Agranulocytosis / Q000188:drug therapy* / Q000209:etiology*; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D009173:Mycophenolic Acid / Q000031:analogs & derivatives* / Q000627:therapeutic use; D012859:Sjogren's Syndrome / Q000150:complications*
chemical_list: D007166:Immunosuppressive Agents; D009173:Mycophenolic Acid