title: TAFRO syndrome with primary Sjogren's syndrome.
abstract: A 25-year-old woman diagnosed 1 year earlier with Primary Sjogren's syndrome was admitted to a nearby hospital with fever of unknown origin. Examination revealed anasarca, systemic lymphadenopathy, hepatosplenomegaly and high C-reactive protein level. The patient's symptoms were initially suspected to be caused by severe bacterial infection with Sjogren's syndrome flare. She was given antibiotics and prednisolone (PSL) at 50 mg/day. However, the patient developed anemia and thrombocytopenia and was transferred to our hospital for further care. Histological examination of the right inguinal lymph node showed neutrophilic infiltration. Bone marrow aspiration revealed a normocellular marrow with increased megakaryocytes and mild reticulin fiber hyperplasia. After initiation of minocycline hydrochloride, the patient's symptoms improved. However, as PSL was tapered, her symptoms worsened. The patient's clinical symptoms and laboratory data improved again with initiation of intravenous steroid pulse therapy and cyclosporine. TAFRO syndrome is characterized by a constellation of symptoms: Thrombocytopenia, Anasarca, Fever, Reticulin fibrosis and Organomegaly. Although histological findings of the lymph node in this case differed from previous reports, the patient's other symptoms and clinical course were similar to TAFRO syndrome. TAFRO syndrome can occur with several diseases, including infection, rheumatic disease and malignancies. We report a case in which infection might have triggered TAFRO syndrome.
authors: Departments of General Internal Medicine and Rheumatology, Nagasaki Medical Center.
authors: Nozomi
authors: N
authors: Iwanaga
authors: Kohei
authors: K
authors: Harada
authors: Yoshika
authors: Y
authors: Tsuji
authors: Chieko
authors: C
authors: Kawahara
authors: Kazuhiro
authors: K
authors: Kurohama
authors: Yasumori
authors: Y
authors: Izumi
authors: Shinichiro
authors: S
authors: Yoshida
authors: Keita
authors: K
authors: Fujikawa
authors: Masahiro
authors: M
authors: Ito
authors: Atsushi
authors: A
authors: Kawakami
authors: Kiyoshi
authors: K
authors: Migita
mesh_terms: D000328:Adult; D001424:Bacterial Infections; D001853:Bone Marrow / Q000473:pathology; D016572:Cyclosporine / Q000627:therapeutic use; D004487:Edema / Q000188:drug therapy / Q000209:etiology*; D005260:Female; D005334:Fever / Q000188:drug therapy / Q000209:etiology*; D005355:Fibrosis / Q000188:drug therapy / Q000209:etiology*; D006801:Humans; D008198:Lymph Nodes / Q000473:pathology; D008199:Lymphadenitis / Q000209:etiology / Q000382:microbiology / Q000473:pathology; D016878:POEMS Syndrome / Q000188:drug therapy / Q000209:etiology*; D012155:Reticulin; D012859:Sjogren's Syndrome / Q000150:complications* / Q000473:pathology; D013577:Syndrome; D013921:Thrombocytopenia / Q000188:drug therapy / Q000209:etiology*; D016896:Treatment Outcome
chemical_list: D012155:Reticulin; D016572:Cyclosporine