[威斯科特-奥尔德里奇综合征患者异基因骨髓移植后的血小板功能]
[Platelet function after allogeneic bone marrow transplantation in Wiskott-Aldrich syndrome].
作者信息
Megido M, Cesar J M, Maldonado M S, Otheo E, Muñoz A, Navarro J L
机构信息
Servicio de Hematología, Hospital Ramón y Cajal, Madrid.
出版信息
Sangre (Barc). 1993 Jun;38(3):233-4.
PURPOSE
To assess the platelet characteristics and functionalism in the Wiskott-Aldrich syndrome (WAS) after allogeneic BMT using cyclophosphamide and busulphan for conditioning.
MATERIAL AND METHODS
Two WAS patients underwent allogeneic BMT. Platelet aggregation was studied prior to and after BMT, along with the intraplatelet amount of ADP and ATP.
RESULTS
Platelet count, size and aggregation wholly recovered after BMT. The post-transplant content of platelet nucleotides was normal.
CONCLUSIONS
Platelet function can be totally restored with cyclophosphamide/busulphan conditioned BMT in WAS. Platelet defects in this disease are due to defective thrombopoiesis.
目的
评估使用环磷酰胺和白消安进行预处理的异基因骨髓移植（BMT）后，维斯科特-奥尔德里奇综合征（WAS）患者的血小板特征及功能。
材料与方法
两名WAS患者接受了异基因BMT。在BMT前后研究了血小板聚集情况，以及血小板内ADP和ATP的含量。
结果
BMT后血小板计数、大小和聚集完全恢复。移植后血小板核苷酸含量正常。
结论
在WAS患者中，使用环磷酰胺/白消安预处理的BMT可使血小板功能完全恢复。该疾病中的血小板缺陷是由于血小板生成缺陷所致。
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