Title: Hematopoietic stem cell transplantation for primary lymphoid immunodeficiencies

标题：造血干细胞移植治疗原发性淋巴祥免疫缺损

Content:

1) Hematopoietic stem cell (HSC) transplantation is curative therapy for many primary immunodeficiencies.

造血干细胞移植能治愈多种原发性免疫缺陷。

2) All forms of severe combined immune deficiency (SCID) can be cured, but the extent of the immunologic correction is dependent on the pathophysiology of the primary defect.

所有类型的重度联合免疫缺陷病均能治愈，免疫纠正的程度取决于原发性缺陷的病理生理学。

3) Defects involving lymphocyte differentiation are more easily corrected than defects in lymphocyte function because a selective advantage exists for the progeny of the normal donor HSC when the primary defect affects lymphocyte differentiation.

纠正淋巴细胞分化相关的缺陷要比淋巴细胞功能缺陷容易，这是因为当原发性缺陷影响淋巴细胞分化时，正常供体HSC的子代有选择优势。

4) T-cell-depleted (TCD), haploidentical-HSC transplantation can cure many forms of SCID, but not other primary immunodeficiencies like the Wiskott-Aldrich syndrome (WAS).

去T细胞的单倍体-HSC移植能治愈多种形式的SCID，但是不包括如 Wiskott-Aldrich综合征(WAS)等原发性免疫缺陷。

5) Unrelated bone marrow and umbilical cord blood are alternative sources of HSC for patients who do not have histocompatible donors.

若患者无组织相容性供体非亲属的骨髓与脐带血是HSC的替代来源。

责任编辑：admin