63例骨髓增生异常综合征的诊断及临床特征分析

Diagnosis and Analysis of Clinical Characteristics of 63 Patients with Myelodysplastic Syndromes

【目的】重新评价68例曾被诊断为骨髓增生异常综合征（MDS）病例其诊断的准确性；同时探讨MDS的临床特征、疗效及转归。【方法】①依据MDS的诊断标准、FAB、及WHO的分型标准评价原诊断；②从症状、体征、血液学、骨髓、治疗效果、疾病转归等0if,床特征做回顾性分析。【结果】5例误诊，确诊63例。男女比例〈1，平均发病年龄56．5岁。难治性贫血（RA）52例（82．5％），环形铁粒细胞性难治性贫血（RAS）1例（1．6％），未成熟细胞过多性难治性贫血（RAEB）8例（12．7％），MDS／难治性血细胞减少伴多系发育异常（RCMD）2例（3．2％）。白血病6例，发生率9．5％。传统方法治疗有效率32％，沙利度胺治疗有效率50％，但两者无统计学差异（P〉0．05）。合并自身免疫性疾病6例，占9．5％，激素治疗后4例部分缓解，有效率67％。RA52倒，转白2例，转白率3．8％，RAEB8例，转白血病4例，转白率50％，两者比较有统计学差异（P〈0．001）。前者平均转白时间38．5个月，后者平均转白时间22．25个月。【结论】RAEB转白率明显高于RA；合并免疫性疾病者激素疗效好；传统治疗与沙利度胺治疗效果比较无统计学意义。

[Objective] To reevaluation the accuracy of the diagnosis of 68 cases diagnosed as myelodysplastic syndrome（MDS） and explore the clinical characteristics, efficacy and prognosis of MDS. [Methods] Based on diagnostic criteria for MDS, FAB and WHO classification criteria, the original diagnosis was evaluated. The symptoms, signs, hematology, bone marrow, therapeutic effect, prognosis and other clinical features were analyzed retrospectively. [Results] Five cases were misdiagnosed and 63 cases were diagnosed. The ratio of male to female was lower than 1, and the average age was 56.5 years old. There were 52 cases of RA （82.5%）, one case of RAS（1.6%）, 8 cases of RAEB（12.7%） and 2 cases of MDS/RCMD（3.2%）. There were 6 patients with leukemia, and the occurrence rate was 9.5%. The effective rate of traditional treatment was 32 %, and that of thalidomide treatment was 50 %, hut there was no significant difference（ P 〉0.05）. Six cases（9.5%） were complicated with autoimmune diseases. Four cases had partial remissions after hormone therapy, and the effective rate was 67%. Two cases of 52 RA cases turned to be leukemia, and the turnover rate was 3.8%. Four cases of 8 RAEB cases turned to be leukemia, and the turnover rate was 50%. There was statistical difference between them（ P 〈0. 001）. The average time for the turnover of the former was 38. 5 months, and that of the latter was 22.25 months. [Conclusion] The rate of RAEB turning to be leukemia is significantly higher than that of RA with statistical significance. Hormone therapy is effective for patients complicated with immunological diseases. There is no significant difference in the therapeutic effect between conventional treatment and thalidomide therapy.