Litzow M R, Kyle R A
Division of Hematology and Internal Medicine, Mayo Clinic Rochester, Minnesota 55905.
Am J Hematol. 1989 Nov;32(3):226-9. doi: 10.1002/ajh.2830320313.
A 55-year-old white woman presented in July 1984 with severe aplastic anemia refractory to anti-thymocyte globulin, corticosteroids, and danazol. In December 1984, oral cyclosporine therapy was begun, and a partial remission was achieved with persistent thrombocytopenia and transfusion independence. The cyclosporine dosage was tapered and then stopped in May 1986 when the platelet count was 35,000/L. In October 1986 the platelet count was only 16,000/L, and the bone marrow showed evidence of a myelodysplastic syndrome; cytogenetic analysis revealed deletion of the long arm of chromosome 13 (previous cytogenetic findings had been normal). After cyclosporine therapy was resumed, platelet count promptly increased to 36,000/L. A subsequent attempt to taper the cyclosporine dosage resulted in a decreased platelet count. The platelet count responded to cyclosporine again and was maintained at 54,000/L and higher with cyclosporine therapy at only 25 mg/day. This information suggests that, despite the development of a myelodysplastic syndrome, immune mechanisms were still operative in the pathogenesis of our patient's thrombocytopenia. Immune modulation may have an important role in preventing progression of myelodysplastic syndromes to more severe forms or to acute leukemia.
一名55岁的白人女性于1984年7月就诊，患有严重再生障碍性贫血，对抗胸腺细胞球蛋白、皮质类固醇和达那唑治疗无效。1984年12月开始口服环孢素治疗，实现了部分缓解，但仍存在持续性血小板减少且无需输血。1986年5月，当血小板计数为35,000/L时，环孢素剂量逐渐减少并停药。1986年10月，血小板计数仅为16,000/L，骨髓显示有骨髓增生异常综合征的迹象；细胞遗传学分析显示13号染色体长臂缺失（之前的细胞遗传学检查结果正常）。重新开始环孢素治疗后，血小板计数迅速升至36,000/L。随后试图减少环孢素剂量导致血小板计数下降。血小板计数对环孢素再次产生反应，通过仅25毫克/天的环孢素治疗，血小板计数维持在54,000/L及以上。这一信息表明，尽管出现了骨髓增生异常综合征，但免疫机制在我们患者血小板减少的发病机制中仍起作用。免疫调节可能在预防骨髓增生异常综合征进展为更严重形式或急性白血病方面具有重要作用。
