A case report of Coombs' test-negative Evans syndrome associated with subclinical Sjögren's syndrome
スポンサーリンク
概要
- 論文の詳細を見る
We describe a 15 year-old girl with autoimmune hemolytic anemia and thrombocytopenic purpura (Evans syndrome) associated with subclinical Sjögren's syndrome.On admission, she was anemic (Hb 7.1g/dl, reticulocytes 144‰) and had jaundice (T. bil. 3.2mg/dl, D. bil. 0.2mg/dl). Petechial hemorrhages were demonstrated on her lower extremities and chest wall. Platelet count was 2.0×104/μl, and serum LDH was increased (798U/l). Increased megakaryocytes and erythroid hyperplasia were identified in the bone marrow. Serum Fe was 77μg/dl, and haptoglobin was markedly decreased (5mg/dl). Both direct and indirect Coombs' tests were continuously negative. ANA was positive at 1:80 (speakled pattern), but anti-DNA antibody and anti-ENA antibody were negative. Anti SS-A antibody was possitive at 1:16, but anti SS-B antibody was negative. Ham test, sugar water test and Donath-Landsteiner antibodies were all negative. Osmotic fragility test, RBC enzymes and Hemoglobin analysis were normal. RBC life span (51Cr-method) was 7.3days (normal: 25_??_35days). Parotid sialography revealed diffuse sialectasis, and both lacrimal and salivary secretions determined by Schirmer test or Gum test were decreased. Keratoconjunctivitis sicca was demonstrated by the rose-bengal test.She was diagnosed as Evans syndrome with Sjögren's syndrome, and treated with prednisolone, 40mg daily. Both hemolytic anemia and thrombocytopenia responded to the treatment.This is the first case report of Coombs' test-negative Evans syndrome associated with Sjögren's syndrome.
- 日本臨床免疫学会の論文
著者
関連論文
- 運動性無緊張性膀胱をはじめとする多彩な中枢・末梢神経障害を示した全身性エリテマトーデスの1例
- 380 胸腺ならびに胸腺腫での癌遺伝子(c-myc, c-Ki-ras, c-Ha-ras)の発現
- 著明な肺高血圧を合併した悪性関節リウマチ(MRA)の1症例 : 日本循環器学会第144回関東甲信越地方会
- 吸収不良症候群と腸管嚢腫様気腫による気腹を伴った全身強皮症の1治験例
- 424 再発性胸腺腫の癌遺伝子蛋白発現に関する研究
- 390 重症筋無力症胸腺の接着蛋白に関する研究
- 55 慢性関節リウマチに対するsulphasalazine(Salazopyrin[○!R])の長期治療成績 : 経口金剤(auranofin)との比較
- 54 慢性関節リウマチ、全身性エリテマトーデス、ベーチェット病患者の末梢血、関節液中の腫瘍壊死性因子(TNF-α)
- 131 全身性エリテマトーデスにおける抗燐脂質抗体の臨床的意義
- 373 シェーグレン症候群患者のNatural Killer細胞