Shimmyozu K, Tara M, Okadome T, Maruyama Y, Maruyama I, Osame M
Third Department of Internal Medicine, Faculty of Medicine, Kagoshima University.
Rinsho Ketsueki. 1990 Sep;31(9):1468-73.
We report a case of pure red cell aplasia (PRCA) with benign monoclonal gammopathy (BMG) of IgA.lambda type and type I von Willebrand disease (vWD). A 61-year-old female patient was treated initially with prednisolone, azathioprine and cyclophosphamide with transient and unsatisfactory reticulocyte response. Oral administration of 200 mg of cyclosporine A (CyA)/day was started from July, 1987. A rapid and marked reticulocytosis was seen from a week later and there was a rapid increase in hemoglobin levels, and remission has been maintained for over 22 months. Patient's serum and IgA taken on admission did not show inhibitory activity to both CFU-E growth from her own bone marrow cells obtained in remission and von Willebrand factor. T cell-mediated suppression to CFU-E growth was detected. On family study, the patient's second son was found to be a type I vWD. These results indicate that there is no direct causal relationships between BMG and PRCA or vWD, and that CyA may have a place in the management of PRCA.
我们报告一例伴有IgA.λ型良性单克隆丙种球蛋白病（BMG）和I型血管性血友病（vWD）的纯红细胞再生障碍性贫血（PRCA）。一名61岁女性患者最初接受泼尼松龙、硫唑嘌呤和环磷酰胺治疗，网织红细胞反应短暂且不理想。1987年7月开始口服200mg/天的环孢素A（CyA）。一周后出现快速且显著的网织红细胞增多，血红蛋白水平迅速升高，缓解状态已维持超过22个月。患者入院时的血清和IgA对其缓解期获得的自身骨髓细胞的CFU - E生长及血管性血友病因子均未显示抑制活性。检测到T细胞介导的对CFU - E生长的抑制作用。在家族研究中，发现患者的次子为I型vWD。这些结果表明BMG与PRCA或vWD之间不存在直接因果关系，且CyA在PRCA的治疗中可能占有一席之地。
