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首页> 外文期刊>Hong Kong Journal of Paediatrics >Haematopoietic Stem Cell Transplant for Wiskott-Aldrich Syndrome
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Haematopoietic Stem Cell Transplant for Wiskott-Aldrich Syndrome

机译：Wiskott-Aldrich综合征的造血干细胞移植

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We reviewed retrospectively 7 Chinese children diagnosed with Wiskott-Aldrich syndrome (WAS) and managed at the Department of Paediatrics & Adolescent Medicine of Queen Mary Hospital from 1988 to 2005. All patients presented with the classical triad of bleeding tendency, recurrent infections and infantile eczema from neonatal period to 2-3 months of age. The median lag time between diagnosis and presentation was 7 months. Thrombocytopenia and small platelet volume were consistent findings and present in all patients. Findings in immunoglobulin level, lymphocyte subset and lymphocyte proliferative studies were heterogeneous. Four mutations were found in 5 (2 cousins shared the same mutation). Haematopoietic stem cell transplant (HSCT) had been performed for all patients. All 7 had complete immune reconstitution with no major long-term complications in a median follow-up of 9.3 years. Early diagnosis and selection of appropriate donor for HSCT were important strategies for improved survival of patients with WAS.
机译：我们回顾性研究了1988年至2005年在玛丽医院（Queen Mary Hospital）儿科和青少年医学科接受诊治并诊断为Wiskott-Aldrich综合征（WAS）的7名中国儿童。所有患者均表现出典型的出血倾向，反复感染和婴儿湿疹三联征。从新生儿期到2-3个月大。从诊断到就诊之间的中位滞后时间为7个月。血小板减少症和小血小板量是一致的发现，并存在于所有患者中。免疫球蛋白水平，淋巴细胞亚群和淋巴细胞增殖研究的发现是异质的。在5个中发现了4个突变（2个堂兄共享相同的突变）。已对所有患者进行了造血干细胞移植（HSCT）。所有7例患者均进行了完全的免疫重建，中位随访9.3年，无重大的长期并发症。早期诊断和选择合适的HSCT供体是提高WAS患者生存率的重要策略。

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