骨髓增生异常综合征112例临床分析

CLINICAL ANALYSIS OF 112 CASES OF MYELODYSPLASTIC SYNDROME

目的：临床研究和分析骨髓增生异常综合征（ＭＤＳ）１１２例。方法：总结自１９８２年１０月至１９９７年１０月期间ＭＤＳ１１２例的一般情况、临床和实验室检查特点及治疗。结果：５５例（４９％）在４０岁以下，１１１例（９９．１％）和９５例（８４％）分别有贫血和全血细胞减少，全部病例骨髓红系有巨幼样变，粒系病态造血达９２％，骨髓活检发现不成熟前体细胞异常定位（ＡＬＩＰ）者为３９％（１４／３６），难治性贫血（ＲＡ）和难治性贫血伴环状铁粒幼细胞（ＲＡＳ）型病人用雄性激素治疗后７８％（６６／８５）血红蛋白有提高，而血小板无明显变化，原始细胞增多型难治性贫血、转化中的原始细胞增多型难治性贫血和慢性粒单细胞白血病型病人主要死于急性白血病。结论：骨髓病态造血和ＡＬＩＰ对诊断有重要价值，雄性激素可改善ＲＡ和ＲＡＳ型病人的贫血。

Objective: To study and analyse clinically 112 cases of myelodysplastic syndromes (MDS) Methods: The general condition, clinical and laboratory features, and therapy of these patients from Oct, 1982 to Oct, 1997 were studied. Results:55 cases (49%) were less than 40 years old.111 cases (99.1%) and 95 cases (84%) developed anemia and pancytopenia respectively at diagnosis. Bone marrow aspirate smears showed megaloblastoid erythropoiesis in all patients and granulocytic dysplasia in 92% of these patients, respectively. Bone marrow biopsy specimens showed ALIP (abnormal localization of immature precursors) in 39%(14/36) of patients. The level of hemoglobin in 78% (66/85) of patients with refractory anemia (RA) and refractory anemia with ringed sideroblasts (RAS) was increased during androgen therapy, but platelet count was not. The most patients with refractory anemia with excess of blasts, refractory anemia with excess of blasts in transformation and chronic myelomonocytic leukemia died in acute leukemia. Conclusion: Bone marrow cellular morphologic dysplasia and ALIP on bone marrow biopsy are important for diagnosis of MDS. Anemia in patients with RA and RAS can be improved by androgen therapy, but how to protect against transformation from MDS to acute leukemia is going to be further studied.