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主要血型不合骨髓移植后纯红细胞再生障碍性贫血的临床观察

Clinical investigation on pure red cell aplasia following major ABO-incompatible bone marrow transplantation.
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摘要 目的 探讨主要血型不合骨髓移植(MIc- BMT)后的纯红细胞再生障碍性贫血(PRCA) 的临床及实验室特点。方法 对3 例MIc- BMT 后PRCA 的临床及实验室有关资料进行分析,并与9 例对照组(8 例血型相合,1 例次要血型不合) 进行比较。结果 3 例PRCA发生于MIc- BMT后8 周内,伴有较高的血型凝集素滴度;未治疗的PRCA 骨髓中幼红细胞比例在0.05 左右,网织红细胞计数≤0.001 ;骨髓植活时间、血小板数、骨髓中粒细胞与巨核细胞在MIc- BMT 与对照组间无明显差异。MIc- BMT 后8 周内输血量明显增多。结论 MIc- BMT 后PRCA有显著的临床与实验室特点,其发生的主要机理可能为受者血型凝集素对供者红系前体细胞的抑制作用。 Objective To explore the characteristics of pure red cell aplasia (PRCA) following major blood-group-incompatible bone marrow transplantation (MIc-BMT).Method Clinical and laboratory features of three cases of PRCA post-MIc-BMT were analyzed and compared with data from 9 cases of control group.Results 3 cases of PRCA occured within 8 weeks post-BMT accompanied by high titers of anti-A or/and anti-B agglutinins.Untreated PRCA had low percentage of erythropoiesis (about 0.05)in bone marrow and very low number(upto 0 001)of reticulocytes in peripheral blood.MIc-BMT and BMT of control group did not differ significantly with respect to the time of engraftment,myelopoiesis and megakaryocytes in marrow and the number of platelets in blood.In comparison with the control group,the MIc-BMT needed greater erythrocyte transfusion surpport with in 8 weeks post-transplant.Conclusion PRCA following MIc-BMT had clinical and laboratory features which were different from those of the control.It was concluded that the mechanism of PRCA in the setting of major ABO-incompatible BMT would contribute to persistently elevated titers of blood group agglutinins which impaired the erythroid precursors. carcinoma and normal stomach tissue had similar molecular weights and isoelectric points,same N terminal amino acid (arginine),same antigenicity by immunodiffuse tests.Their activities could be inhibited by p chloramercuric benzoic acid,and partially restored by dithiothreital.But ADA in stomach carcinoma had much higher Km and poorer thermostability compared with those in normal tissue. Key words: adenosine deaminase; carcinoma/enzymology; stomach/enzymology
出处 《中国实用内科杂志》 CAS CSCD 北大核心 1999年第10期602-604,共3页 Chinese Journal of Practical Internal Medicine
关键词 骨髓移植 血型不合 纯红细胞 再生障碍性 贫血 Bone marrow transplantation Blood group incompatibility Major pure red cell aplasia
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