Section of Pediatric Allergy/ Immunology, Department of Pediatrics, King Faisal Specialist Hospital & Research Center, MBC-58 3354, Riyadh, 11211, Saudi Arabia.
Colleges of Medicine, Alfaisal University, Riyadh, Saudi Arabia.
J Clin Immunol. 2019 May;39(4):414-420. doi: 10.1007/s10875-019-00634-3. Epub 2019 Apr 30.
One of the limiting factors for successful hematopoietic stem cell transplantation (HSCT) is graft versus host disease (GVHD). The EBMT/ESID guidelines for HSCT in severe combined immunodeficiency (SCID) recommend no GVHD prophylaxis for a matched sibling donor (MSD).
To determine the risk of GVHD in MSD HSCT for SCID patients compared to matched related donor (MRD).
This retrospective cohort study compares MSD with MRD and the outcome of GVHD in all SCID patients who underwent HSCT between 1993 and 2013. All statistical analyses were done using IBM SPSS statistics software.
One hundred forty-five SCID patients underwent 152 HSCTs while 82 (54%) received GVHD prophylaxis. GVHD occurred in 48 patients (31.5%); 20/48 (42%) had GVHD prophylaxis compared to 28/48 (58%) that did not, P = 0.022. Acute GVHD occurred at a higher trend in MSD, 37/120 (30.8%), compared to MRD, 6/32 (18.8%), P = 0.17. We also analyzed the outcome according to the period of HSCT. The first period was 1993 to 2003, 48 HSCTs, 43 MSD, 5 MRD; all patients had GVHD prophylaxis, and there was no difference in GVHD. The second period was 2004 to 2013: of 104 HSCTs, 77 had MSD and 27 had MRD; GVHD prophylaxis was used in 22.1% of MSD and 63% of MRD, P = 0.000. GVHD was significantly higher in the MSD (40.2%) compared to MRD (18.5%) patients, P = 0.041.
GVHD prophylaxis in MSD transplant should be considered in SCID patients.
造血干细胞移植(HSCT)成功的限制因素之一是移植物抗宿主病(GVHD)。EBMT/ESID 关于严重联合免疫缺陷(SCID)HSCT 的指南建议,对于匹配的同胞供体(MSD),不进行 GVHD 预防。
确定与匹配相关供体(MRD)相比,MSD HSCT 治疗 SCID 患者的 GVHD 风险。
本回顾性队列研究比较了 MSD 与 MRD,并比较了 1993 年至 2013 年间所有接受 HSCT 的 SCID 患者的 GVHD 结局。所有统计分析均使用 IBM SPSS statistics 软件进行。
145 例 SCID 患者接受了 152 次 HSCT,其中 82 例(54%)接受了 GVHD 预防。48 例患者发生 GVHD(31.5%);48 例中有 20 例(42%)进行了 GVHD 预防,而 48 例中有 28 例(58%)没有进行预防,P=0.022。MSD 中急性 GVHD 的发生率较高,为 37/120(30.8%),而 MRD 中为 6/32(18.8%),P=0.17。我们还根据 HSCT 的时间段分析了结果。第一个时间段为 1993 年至 2003 年,共进行了 48 次 HSCT,其中 43 次为 MSD,5 次为 MRD;所有患者均接受 GVHD 预防,且 GVHD 无差异。第二个时间段为 2004 年至 2013 年:共进行了 104 次 HSCT,其中 77 次为 MSD,27 次为 MRD;MSD 中有 22.1%的患者接受了 GVHD 预防,而 MRD 中有 63%的患者接受了预防,P=0.000。MSD 组(40.2%)的 GVHD 发生率明显高于 MRD 组(18.5%),P=0.041。
对于 SCID 患者,MSD 移植时应考虑进行 GVHD 预防。