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性别与严重再生障碍性贫血患者对抗淋巴细胞球蛋白(ALG)治疗的反应

Gender and response to antilymphocyte globulin (ALG) for severe aplastic anaemia.

作者信息

Nissen C, Gratwohl A, Tichelli A, Stebler C, Würsch A, Moser Y, Dalle Carbonare V, Signer E, Buser M, Ritz R

机构信息

Department of Research, University Hospital Basel, Switzerland.

出版信息

Br J Haematol. 1993 Feb;83(2):319-25. doi: 10.1111/j.1365-2141.1993.tb08288.x.

Abstract

We have evaluated the speed of haematological recovery in 103 severe aplastic anaemia (SAA) patients treated with antilymphocyte globulin (ALG) and followed at our institution for 3-15 years. We found that haemopoietic recovery was significantly delayed in six girls under the age of 10 years. This slow recovery in girls might be explained by their relative inability to release haemopoietic growth factors, granulocyte colony stimulating activity and burst promoting activity, compared to all other sex and age groups. This defect is not explained by disease severity at presentation and thus indicates a functional abnormality of monocytes/macrophages and T-lymphocytes in addition to the deficiency of haemopoietic stem cells. In a multivariate analysis, low factor production and low pretreatment reticulocyte counts turned out to be strong predictors of slow haemopoietic recovery. We conclude that young girls have a particular form of SAA characterized by low haemopoietic factor production and delayed recovery after ALG. They are preferential candidates for early bone marrow transplantation or, if they are not eligible, for treatment with recombinant human haemopoietic growth factors.

摘要

我们评估了103例接受抗淋巴细胞球蛋白(ALG)治疗并在我们机构随访3至15年的重型再生障碍性贫血(SAA)患者的血液学恢复速度。我们发现,10岁以下的6名女孩的造血恢复明显延迟。与所有其他性别和年龄组相比,女孩造血恢复缓慢可能是由于她们相对无法释放造血生长因子、粒细胞集落刺激活性和爆式促进活性。这种缺陷无法用初诊时的疾病严重程度来解释,因此除了造血干细胞缺乏外,还表明单核细胞/巨噬细胞和T淋巴细胞存在功能异常。在多变量分析中,低因子产生和低预处理网织红细胞计数被证明是造血恢复缓慢的强预测指标。我们得出结论,年轻女孩患有一种特殊形式的SAA,其特征是造血因子产生低且ALG治疗后恢复延迟。她们是早期骨髓移植的优先候选者,或者,如果不符合条件,则是重组人造血生长因子治疗的候选者。

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