Primary immunodeficiency disorders (PIDs) result from inborn errors in immunity.Susceptibility to infections and oftentimes severe autoimmunity pose life-threatening risks to patients with these disorders.Hematopoieti...Primary immunodeficiency disorders (PIDs) result from inborn errors in immunity.Susceptibility to infections and oftentimes severe autoimmunity pose life-threatening risks to patients with these disorders.Hematopoietic cell transplant (HCT) remains the only curative option for many.Severe combined immunodeficiency disorders (SCID) most commonly present at the time of birth and typically require emergent HCT in the first few weeks of life.HCT poses an unusual challenge for PIDs.Donor source and conditioning regimen often impact the outcome of immune reconstitution after HCT in PIDs.The use of matched or unmatched, as well as related versus unrelated donor has resulted in variable outcomes for different subsets of PIDs.Additionally, there is significant variability in the success of engraftment even for a single patient’s lymphocyte subpopulations.While certain cell lines do well without a conditioning regimen, others will not reconstitute unless conditioning is used.The decision to proceed with a conditioning regimen in an already immunocompromised host is further complicated by the fact that alkylating agents should be avoided in radiosensitive PIDs.This manuscript reviews some of the unique elements of HCT in PIDs and evidence-based approaches to transplant in patients with these rare and challenging disorders.展开更多
全身放疗(Total body irradiation,TBI)是对患者全身进行照射的一种特殊放疗技术,是血液系统疾病行造血干细胞移植前的一种预处理手段,其主要作用包括杀灭残存肿瘤细胞、抑制机体免疫应答、为造血干细胞移植排空骨髓空间等。目前,全身...全身放疗(Total body irradiation,TBI)是对患者全身进行照射的一种特殊放疗技术,是血液系统疾病行造血干细胞移植前的一种预处理手段,其主要作用包括杀灭残存肿瘤细胞、抑制机体免疫应答、为造血干细胞移植排空骨髓空间等。目前,全身放疗实施的主要实现方式包括长距离固定野照射、容积旋转调强和分段照射。在本文中,我们结合自身临床实践经验,综述了上述这些实现方式的技术特点和相关研究进展。展开更多
文摘Primary immunodeficiency disorders (PIDs) result from inborn errors in immunity.Susceptibility to infections and oftentimes severe autoimmunity pose life-threatening risks to patients with these disorders.Hematopoietic cell transplant (HCT) remains the only curative option for many.Severe combined immunodeficiency disorders (SCID) most commonly present at the time of birth and typically require emergent HCT in the first few weeks of life.HCT poses an unusual challenge for PIDs.Donor source and conditioning regimen often impact the outcome of immune reconstitution after HCT in PIDs.The use of matched or unmatched, as well as related versus unrelated donor has resulted in variable outcomes for different subsets of PIDs.Additionally, there is significant variability in the success of engraftment even for a single patient’s lymphocyte subpopulations.While certain cell lines do well without a conditioning regimen, others will not reconstitute unless conditioning is used.The decision to proceed with a conditioning regimen in an already immunocompromised host is further complicated by the fact that alkylating agents should be avoided in radiosensitive PIDs.This manuscript reviews some of the unique elements of HCT in PIDs and evidence-based approaches to transplant in patients with these rare and challenging disorders.
文摘全身放疗(Total body irradiation,TBI)是对患者全身进行照射的一种特殊放疗技术,是血液系统疾病行造血干细胞移植前的一种预处理手段,其主要作用包括杀灭残存肿瘤细胞、抑制机体免疫应答、为造血干细胞移植排空骨髓空间等。目前,全身放疗实施的主要实现方式包括长距离固定野照射、容积旋转调强和分段照射。在本文中,我们结合自身临床实践经验,综述了上述这些实现方式的技术特点和相关研究进展。
