50例存活三年以上重型再生障碍性贫血患者的长期随访  

A long-term follow-up study of 50 patients with severe aplastic anemia who have survived more than 3 years after immunosuppressive therapy

作　　者：何广胜[1] 邵宗鸿[1] 刘鸿[1] 张益枝[1] 陈桂彬[1] 李克[1] 宋鲁燕[1] 和虹[1] 赵明峰[1] 付蓉[1] 施均[1] 白洁[1] 张泓[1] 储榆林[1] 杨天楹[1] 杨崇礼[1] 

机构地区：[1]中国医学科学院,中国协和医科大学血液学研究所,血液病医院,天津300020

出　　处：《中华血液学杂志》2002年第5期229-232,共4页Chinese Journal of Hematology

基　　金：卫生部基金资助项目 (962 0 17);天津市自然科学基金资助项目(0 0 36 0 6 111)

摘　　要：目的　评价重型再生障碍性贫血 (SAA)患者免疫抑制治疗 (IST)后的远期疗效。方法　对 50例IST后存活 3年以上SAA患者长期随访 ,了解其造血重建 (血常规、骨髓象、骨髓活检、造血祖细胞体外培养 )、T淋巴细胞免疫、生活质量及治疗不良反应等情况。结果　IST后各随访时间 (3年、4年、5年以上 )的患者中分别有 81 .3 % (1 3例 )、86 .7% (1 3例 )、89.5 % (1 7例 )血常规正常 ,93 .4 % (1 5例 )、93 .3 % (1 4例 )、94.7% (1 8例 )骨髓象正常 ,37.5 % (6例 )、40 .0 % (6例 )、73 .7% (1 4例 )造血祖细胞培养正常 ;全部患者中有 86 .0 % (43例 )血常规正常、94.0 % (47例 )骨髓象正常、52 .0 % (2 6例 )造血祖细胞培养正常 ,88.0 % (44例 )Karnofsky评分达 1 0 0分。各随访时间共有 31例患者进行了骨髓活检 ,其中 2 6例 (83 .9% )正常 ;37例患者进行了T淋巴细胞亚群检查 ,其中 2 9例 (78.4 % )正常。未发现晚期克隆性疾病。治疗远期不良反应轻微。 2 4例患者的全部随访检查结果正常。结论　部分SAA患者经IST后 ,造血功能、T淋巴细胞免疫、生活质量可完全恢复 ,且无显著不良反应 。Objective To evaluate the long term outcome of immunosuppressive therapy(IST) in patients with severe aplastic anemia (SAA). Methods Hematopoietic recovery (peripheral blood cell counts, bone marrow aspirates, bone marrow biopsy, in vitro culture of hematopoietic progenitors), immunity of T lymphocyte, quality of life and side effects of the therapy were assessed in 50 SAA patients who have survived more than 3 years after IST. Results At 3 years, 4 years and 5 years follow up, 81.5% (13 cases), 86.7%(13 cases) and 89.5%(17 cases) of the SAA patients reached and maintained normal peripheral blood cell counts, 93.4%(15 cases), 93 3%(14 cases) and 94.7%(18 cases) showed normal bone marrow pictures,and 37.5%(6 cases), 40.0%(6 cases) and 73.7%(14 cases) had normal yields of bone marrow cell culture, respectively. Overall, 86.0%(43 cases), 94.0% (47 cases) and 52.0%(26 cases) of the total SAA patients were normalized in peripheral blood counts, bone marrow picture and culture of hematopoietic progenitor yields, respectively. During the follow up, 88 0%(44 cases) of the patients achieved 100 of Karnofsky scores; 26 of the 31 patients (83.9%) who received bone marrow biopsy showed normal histological pictures,and 29 of 37 patients (78.4%) tested had normal subsets of T lymphocytes. No clonal disease was found. The late side effects of IST were mild. All of the parameters tested were normal in 24 patients. Conclusion After IST, the hematopoietic function of bone marrow, the immunity of the T lymphocyte and the life quality were normalized with few side effects in patients with SAA. These patients would probably be cured.

关 键 词：重型再生障碍性贫血 免疫抑制治疗 远期疗效 造血生长因子 

分 类 号：R556.5[医药卫生—血液循环系统疾病]