TAFRO syndrome with primary Sjogren's syndrome
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- IWANAGA Nozomi
- Departments of General Internal Medicine and Rheumatology, Nagasaki Medical Center
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- HARADA Kohei
- Departments of General Internal Medicine and Rheumatology, Nagasaki Medical Center
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- TSUJI Yoshika
- Departments of General Internal Medicine and Rheumatology, Nagasaki Medical Center
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- KAWAHARA Chieko
- Departments of General Internal Medicine and Rheumatology, Nagasaki Medical Center
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- KUROHAMA Kazuhiro
- Departments of Pathology, Nagasaki Medical Center
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- IZUMI Yasumori
- Departments of General Internal Medicine and Rheumatology, Nagasaki Medical Center
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- YOSHIDA Shinichiro
- Departments of Hematology, Nagasaki Medical Center
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- FUJIKAWA Keita
- Department of Rheumatology, Isahaya Genaral Hospital
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- ITO Masahiro
- Departments of Pathology, Nagasaki Medical Center
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- KAWAKAMI Atsushi
- Department of Rheumatology, Nagasaki University Hospital
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- MIGITA Kiyoshi
- Departments of General Internal Medicine and Rheumatology, Nagasaki Medical Center
Bibliographic Information
- Other Title
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- TAFRO症候群類似の臨床像を呈した原発性シェーグレン症候群の1例
- TAFRO syndrome with primary Sjögren's syndrome
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Abstract
<p> 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.</p>
Journal
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- Japanese Journal of Clinical Immunology
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Japanese Journal of Clinical Immunology 39 (5), 478-484, 2016
The Japan Society for Clinical Immunology