Bandar Al-Saud1,2, Alhanouf Al-Saleem3, Bashayer Al Rasheed3, Abdulaziz Al-Ghonaium3, Ali Al-Ahmari4,5, Hamoud Al-Mousa3,4, Amal Al-Seraihy5, Rand Arnaout3,4, Abdullah Al-Jefri5, Sahar Elshorbagi3, Nazeema Elsayed6, Hasan Al-Dhekri3, Mouhab Ayas5, Saleh Al-Muhsen3,7. 1. Section of Pediatric Allergy/ Immunology, Department of Pediatrics, King Faisal Specialist Hospital & Research Center, MBC-58 3354, Riyadh, 11211, Saudi Arabia. balsaud@kfshrc.edu.sa. 2. Colleges of Medicine, Alfaisal University, Riyadh, Saudi Arabia. balsaud@kfshrc.edu.sa. 3. Section of Pediatric Allergy/ Immunology, Department of Pediatrics, King Faisal Specialist Hospital & Research Center, MBC-58 3354, Riyadh, 11211, Saudi Arabia. 4. Colleges of Medicine, Alfaisal University, Riyadh, Saudi Arabia. 5. Department of Pediatric Hematology/Oncology, King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia. 6. Nursing affairs, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia. 7. Department of Pediatrics, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
Abstract
BACKGROUND: 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). OBJECTIVE: To determine the risk of GVHD in MSD HSCT for SCID patients compared to matched related donor (MRD). METHODS: 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. RESULTS: 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. CONCLUSION: GVHD prophylaxis in MSD transplant should be considered in SCID patients.
BACKGROUND: 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). OBJECTIVE: To determine the risk of GVHD in MSD HSCT for SCIDpatients compared to matched related donor (MRD). METHODS: This retrospective cohort study compares MSD with MRD and the outcome of GVHD in all SCIDpatients who underwent HSCT between 1993 and 2013. All statistical analyses were done using IBM SPSS statistics software. RESULTS: One hundred forty-five SCIDpatients 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. CONCLUSION:GVHD prophylaxis in MSD transplant should be considered in SCIDpatients.
Entities:
Keywords:
Cyclosporine; Graft versus host disease; Hematopoietic stem cell transplantation; Primary immunodeficiency diseases; Severe combined immunodeficiency
Authors: Mohammad I El-Mouzan; Abdullah A Al-Salloum; Abdullah S Al-Herbish; Mansour M Qurachi; Ahmad A Al-Omar Journal: Saudi Med J Date: 2007-12 Impact factor: 1.484
Authors: Corinne Antoine; Susanna Müller; Andrew Cant; Marina Cavazzana-Calvo; Paul Veys; Jaak Vossen; Anders Fasth; Carsten Heilmann; Nicolas Wulffraat; Reinhard Seger; Stéphane Blanche; Wilhelm Friedrich; Mario Abinun; Graham Davies; Robert Bredius; Ansgar Schulz; Paul Landais; Alain Fischer Journal: Lancet Date: 2003-02-15 Impact factor: 79.321
Authors: R H Buckley; S E Schiff; R I Schiff; L Markert; L W Williams; J L Roberts; L A Myers; F E Ward Journal: N Engl J Med Date: 1999-02-18 Impact factor: 91.245
Authors: William T Shearer; Elizabeth Dunn; Luigi D Notarangelo; Christopher C Dvorak; Jennifer M Puck; Brent R Logan; Linda M Griffith; Donald B Kohn; Richard J O'Reilly; Thomas A Fleisher; Sung-Yun Pai; Caridad A Martinez; Rebecca H Buckley; Morton J Cowan Journal: J Allergy Clin Immunol Date: 2013-11-28 Impact factor: 10.793
Authors: Niraj C Patel; Javier Chinen; Howard M Rosenblatt; Imelda C Hanson; Betty S Brown; Mary E Paul; Stuart L Abramson; Jerome Ritz; William T Shearer Journal: J Allergy Clin Immunol Date: 2008-12 Impact factor: 10.793
Authors: Christopher C Dvorak; Morton J Cowan; Brent R Logan; Luigi D Notarangelo; Linda M Griffith; Jennifer M Puck; Donald B Kohn; William T Shearer; Richard J O'Reilly; Thomas A Fleisher; Sung-Yun Pai; I Celine Hanson; Michael A Pulsipher; Ramsay Fuleihan; Alexandra Filipovich; Frederick Goldman; Neena Kapoor; Trudy Small; Angela Smith; Ka-Wah Chan; Geoff Cuvelier; Jennifer Heimall; Alan Knutsen; Brett Loechelt; Theodore Moore; Rebecca H Buckley Journal: J Clin Immunol Date: 2013-07-02 Impact factor: 8.542