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23例重症再生障碍性贫血淋巴细胞去除术的治疗结果

Treatment results of 23 cases of severe aplastic anemia with lymphocytapheresis.

作者信息

Morales-Polanco M R, Sánchez-Valle E, Guerrero-Rivera S, Gutiérrez-Alamillo L, Delgado-Márquez B

机构信息

Facultad de Medicina, Universidad Nacional Autónoma de México, México, D.F.

出版信息

Arch Med Res. 1997 Spring;28(1):85-90.

PMID:9078593
Abstract

We report the results of 23 patients with aplastic anemia (AA) treated with a program of 14 lymphocytapheresis (LC). Treatments were performed with apheresis machines, models Haemonetics 30-S and Baxter CS3000, using the standard program. This procedure was done because AA in many cases appears as a result of the action of a T cell population that inhibits hematopoiesis. Theoretically, removal of this clonal population would produce hematopoietic recovery. Of the total of 23 patients, 9 were excluded for final evaluation of treatment results because 7 died during or shortly after treatment (0.7-3 months); one patient abandoned treatment after three LC and another died 7 months later because of transformation to acute leukemia. The remaining 14 patients were included in the final evaluation of treatment; seven females and seven males, average age 46.1 years (range 22-69); 13 with severe, and one with moderate AA; 11 with recently diagnosed, and 3 with chronic AA; 12 without previous treatment and two treated before with antilymphocyte globulin + oxymetholone (OXM) + cyclosporine A (CsA) with transient partial remission (PR). Besides lymphocytapheresis, 13 patients received OXM; 4 of them GM-CSF and one low dose CsA. Four patients had complete remission lasting > 59.5 months (range 42-78); eight PR (average duration of > 38.6 months), and two minimal remission (> 37 and 29 months). Platelet, reticulocyte and granulocyte counts increased on average at 48.7, 73.3 and 91.4 days, respectively. In conclusion, 14 (60.8%) of 23 patients with AA showed an improvement related to LC treatment, with a survival probability of 63% from the fourth month, the latter with an added beneficial effect of the other therapies used. Larger numbers of patients have to be treated with LC to determine its real usefulness, mechanism of action and the best conditions for its use.

摘要

我们报告了23例再生障碍性贫血(AA)患者采用14次淋巴细胞去除术(LC)治疗方案的结果。使用Haemonetics 30 - S型和百特CS3000型血细胞分离机,按照标准程序进行治疗。进行该操作的原因是,在许多情况下,AA是由抑制造血的T细胞群作用导致的。理论上,去除这种克隆性细胞群会使造血功能恢复。在总共23例患者中,9例被排除在治疗结果的最终评估之外,因为7例在治疗期间或治疗后不久(0.7 - 3个月)死亡;1例患者在进行3次LC后放弃治疗,另1例在7个月后因转化为急性白血病死亡。其余14例患者纳入治疗的最终评估;7例女性和7例男性,平均年龄46.1岁(范围22 - 69岁);13例为重度AA,1例为中度AA;11例为近期诊断,3例为慢性AA;12例未接受过先前治疗,2例之前接受过抗淋巴细胞球蛋白 + 羟甲烯龙(OXM)+ 环孢素A(CsA)治疗并获得短暂部分缓解(PR)。除淋巴细胞去除术外,13例患者接受了OXM治疗;其中4例接受粒细胞 - 巨噬细胞集落刺激因子(GM - CSF)治疗,1例接受低剂量CsA治疗。4例患者获得持续超过59.5个月(范围42 - 78个月)的完全缓解;8例部分缓解(平均持续时间超过38.6个月),2例微小缓解(超过37个月和29个月)。血小板、网织红细胞和粒细胞计数分别平均在48.7、73.3和91.4天增加。总之,23例AA患者中有14例(60.8%)显示与LC治疗相关的改善,从第四个月起生存概率为63%,后者还得益于所使用的其他治疗方法的有益作用。必须对更多患者进行LC治疗,以确定其实际效用、作用机制和最佳使用条件。

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Treatment results of 23 cases of severe aplastic anemia with lymphocytapheresis.23例重症再生障碍性贫血淋巴细胞去除术的治疗结果
Arch Med Res. 1997 Spring;28(1):85-90.
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Immunosuppressive treatment of acquired aplastic anemia and immune-mediated bone marrow failure syndromes.获得性再生障碍性贫血及免疫介导的骨髓衰竭综合征的免疫抑制治疗
Int J Hematol. 2002 Feb;75(2):129-40. doi: 10.1007/BF02982017.