全身性エリテマトーデスに合併しステロイド大量療法が奏功した骨髄線維症の１例

• Published in: 臨床リウマチ Vol. 20; no. 4; pp. 302 - 309
• Main Authors: 遠藤, 平仁, 橋本, 篤, 吉田, 秀, 廣畑, 俊成, 田中, 住明, 飯塚, 進子, 木村, 美保, 近藤, 啓文, 石川, 章, 田中, 淳一
• Format: Journal Article
• Language: Japanese
• Published: 一般社団法人 日本臨床リウマチ学会 2008; The Japanese Society for Clinical Rheumatology and Related Research
• Subjects: myelofibrosis; pancytopenia; systemic lupus erythematosus (SLE)
• ISSN: 0914-8760; 2189-0595
• DOI: 10.14961/cra.20.302

    A woman of 50 years of age who had a 13-year history of hypothyroidism was diagnosed with systemic lupus erythematosus (SLE) with butterfly rash, leukopenia, positivity of antinuclear antibody, anti-DNA antibody and anti-Sm antibody. Two years later, she developed nephritis (WHO type IV) and remitted with corticosteroid pulse and intermittent intravenous cyclophosphamide pulse therapy (IVCY). Four years after the onset of SLE, she relapsed with proteinuria and leukopenia when she was taking 9 mg/day of prednisolone (PSL) but she stopped all the medication of her own accord. Four months passed without any therapy, she was admitted to our hospital with disturbance of consciousness and anasarca. Laboratory findings showed pancytopenia (WBC 1300/μl, RBC 233×10⁴/μl, Hb6.9g/dl, Plt3.6×10⁴/μl), aggravation of lupus nephritis and hypothyroidism. Chest X-ray and ultrasonography demonstrated pleural and pericardial effusion and the absence of hepatosplenomegaly. She was also diagnosed with myelofibrosis upon bone marrow inspection. Three instances of corticosteroid pulse therapy, oral corticosteroid (PSL was tapered from 50 mg/day) and supplement therapy of levothyroxine improved every symptom and pancytopenia. The second bone marrow biopsy showed reduced fibrosis and recovery of bone marrow cells. These findings implied the secondary myelofibrosis caused by SLE because the myelofibrosis came along with aggravation of SLE and corticosteroid therapy was effective. This is a rare case of SLE in which myelofibrosis improved by high-dose corticosteroid therapy, which was confirmed by bone marrow biopsy and suggests the pathogenic mechanisms for myelofibrosis.