Two Successful Bone Functions in Patients with Chronic Granulomatous Disease Marrow Transplantations Improved Lung
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Abstract
Chronic Granulomatous Disease (CGD) is a rare inherited primary immune deficiency disorder with defective respiratory burst activity in phagocytes, resulting in recurrent pyogenic infections. In this study, we described two CGD patients who had done bone marrow transplantation (BMT). As Bone marrow transplantation (BMT) is the definitive treatment of the disease, we evaluated the function of their lungs before and after BMT. In both patients, the BMT was from their siblings. In case 1, the patient’s pulmonary function (PFT) before BMT was: FEV1: 34, FVC: 40, FEV1 / FVC: 72%, and after BMT was: FEV1: 66, FVC: 40 by 49, FEV1 / FVC: 64%. In case 2, the patient’s PFT before BMT was: FEV1: 22, FVC: 36, FEV1 / FVC: 41%, and after BMT was: FEV1: 47, FVC: 33, FEV1/FVC: 43%. BMT significantly improved their Pulmonary Problems and Preclinical (PFT). In addition, after BMT, both patients’ well-tolerated clinical signs and the infection rate, and the number of hospitalizations in both patients decreased.
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[12] Leung T, Chik K, Li C, Shing M, Yuen P. Bone marrow transplan- tation for chronic granulomatous disease: Long-term follow-up and review of literature. Bone Marrow Transplantation. 1999; 24(5):567-70. [DOI:10.1038/sj.bmt.1701932] [PMID]
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[15] Soncini E, Slatter MA, Jones LB, Hughes S, Hodges S, Flood TJ, et al. Unrelated donor and HLA-identical sibling haematopoietic stem cell transplantation cure chronic granulomatous disease with good long- term outcome and growth. British Journal of Haematology. 2009; 145(1):73-83. [DOI:10.1111/j.1365-2141.2009.07614.x] [PMID]
[16] Güngör T, Teira P, Slatter M, Stussi G, Stepensky P, Moshous D, et al. Reduced-intensity conditioning and HLA-matched haemopoi- etic stem-cell transplantation in patients with chronic granuloma- tous disease: A prospective multicentre study. The Lancet. 2014; 383(9915):436-48. [DOI:10.1016/S0140-6736(13)62069-3][PMID]
[17] Srinivasan A, Sunkara A, Mitchell W, Sunthankar S, Kang G, Stokes DC, et al. Recovery of pulmonary function after allogeneic hemat- opoietic cell transplantation in children is associated with improved survival. Biology of Blood and Marrow Transplantation. 2017; 23(12):2102-9. [DOI:10.1016/j.bbmt.2017.08.025] [PMID] [PMCID]
[18] Srinivasan A, Srinivasan S, Sunthankar S, Sunkara A, Kang G, Stokes DC, et al. Pre-Hematopoietic stem cell transplant lung function and pulmonary complications in children. Annals of the American Thoracic Society. 2014; 11(10):1576-85. [DOI:10.1513/ AnnalsATS.201407-308OC] [PMID] [PMCID]
[19] Salvator H, Mahlaoui N, Catherinot E, Rivaud E, Pilmis B, Borie R, et al. Pulmonary manifestations in adult patients with chronic granulomatous disease. European Respiratory Journal. 2015; 45(6):1613-23. [DOI:10.1183/09031936.00118414] [PMID]
[2] Segal AW. How neutrophils kill microbes. Annual Review of Im- munology. 2005; 23:197-223. [DOI:10.1146/annurev.immu- nol.23.021704.115653] [PMID] [PMCID]
[3] Papayannopoulos V, Zychlinsky A. NETs: A new strategy for us- ing old weapons. Trends in Immunology. 2009; 30(11): 513-21. [DOI:10.1016/j.it.2009.07.011] [PMID]
[4] Kuhns DB, Alvord WG, Heller T, Feld JJ, Pike KM, Marciano BE, et al. Residual NADPH oxidase and survival in chronic granulomatous disease. New England Journal of Medicine. 2010; 363(27):2600-10. [DOI:10.1056/NEJMoa1007097] [PMID] [PMCID]
[5] Lublin M, Bartlett DL, Danforth DN, Kauffman H, Gallin JI, Ma- lech HL, et al. Hepatic abscess in patients with chronic granu- lomatous disease. Annals of Surgery. 2002; 235(3):383-91. [DOI:10.1097/00000658-200203000-00010] [PMID] [PMCID]
[6] Roos D, Kuhns DB, Maddalena A, Roesler J, Lopez JA, Ariga T, et al. He- matologically important mutations: X-linked chronic granulomatous disease (third update). Blood Cells, Molecules, and Diseases. 2010; 45(3):246-65. [DOI:10.1016/j.bcmd.2010.07.012] [PMID] [PMCID]
[7] Segal BH, DeCarlo ES, Kwon-Chung KJ, Malech HL, Gallin JI, Hol- land SM. Aspergillus nidulans infection in chronic granulomatous disease. Medicine. 1998; 77(5):345-54. [DOI:10.1097/00005792- 199809000-00004] [PMID]
[8] Vinh DC, Shea YR, Jones PA, Freeman AF, Zelazny A, Holland SM. Chronic invasive aspergillosis caused by aspergillus viridinutans. Emerging Infectious Diseases. 2009; 15(8):1292-4. [DOI:10.3201/ eid1508.090251] [PMID] [PMCID]
[9] Hamidieh AA, Pourpak Z, Yari K, Fazlollahi MR, Hashemi S, Behfar M, et al. Hematopoietic stem cell transplantation with a reduced- intensity conditioning regimen in pediatric patients with griscelli syndrome type 2. Pediatric Transplantation. 2013; 17(5):487-91. [DOI:10.1111/petr.12092] [PMID]
[10] De Ravin SS, Naumann N, Cowen EW, Friend J, Hilligoss D, Marques- en M, et al. Chronic granulomatous disease as a risk factor for auto- immune disease. Journal of Allergy and Clinical Immunology. 2008; 122:1097-103. [DOI:10.1016/j.jaci.2008.07.050] [PMID] [PMCID]
[11] Kang EM, Marciano BE, DeRavin S, Zarember KA, Holland SM, Malech HL. Chronic granulomatous disease: Overview and hemat- opoietic stem cell transplantation. Journal of Allergy and Clinical Im- munology. 2011; 127(6):1319-26. [DOI:10.1016/j.jaci.2011.03.028] [PMID] [PMCID]
[12] Leung T, Chik K, Li C, Shing M, Yuen P. Bone marrow transplan- tation for chronic granulomatous disease: Long-term follow-up and review of literature. Bone Marrow Transplantation. 1999; 24(5):567-70. [DOI:10.1038/sj.bmt.1701932] [PMID]
[13] Rezaei N, Aghamohammadi A, Moin M, Pourpak Z, Movahedi M, Gharagozlou M, et al. Frequency and clinical manifestations of patients with primary immunodeficiency disorders in Iran: Update from the Iranian Primary Immunodeficiency Registry. Journal of Clinical Immunology. 2006; 26(6):519-32. [DOI:10.1007/s10875- 006-9047-x] [PMID]
[14] Rich RR, Fleisher TA, Shearer WT, Frew AJ, Schroeder Jr HW, Weyand CM. Clinical immunology: Principles and practice. Amsterdam: El- sevier; 2013. https://www.google.com/books/edition/Clinical_Im- munology_Principles_and_Pract/MRTa5qwTc7AC?hl=en&gbpv=0
[15] Soncini E, Slatter MA, Jones LB, Hughes S, Hodges S, Flood TJ, et al. Unrelated donor and HLA-identical sibling haematopoietic stem cell transplantation cure chronic granulomatous disease with good long- term outcome and growth. British Journal of Haematology. 2009; 145(1):73-83. [DOI:10.1111/j.1365-2141.2009.07614.x] [PMID]
[16] Güngör T, Teira P, Slatter M, Stussi G, Stepensky P, Moshous D, et al. Reduced-intensity conditioning and HLA-matched haemopoi- etic stem-cell transplantation in patients with chronic granuloma- tous disease: A prospective multicentre study. The Lancet. 2014; 383(9915):436-48. [DOI:10.1016/S0140-6736(13)62069-3][PMID]
[17] Srinivasan A, Sunkara A, Mitchell W, Sunthankar S, Kang G, Stokes DC, et al. Recovery of pulmonary function after allogeneic hemat- opoietic cell transplantation in children is associated with improved survival. Biology of Blood and Marrow Transplantation. 2017; 23(12):2102-9. [DOI:10.1016/j.bbmt.2017.08.025] [PMID] [PMCID]
[18] Srinivasan A, Srinivasan S, Sunthankar S, Sunkara A, Kang G, Stokes DC, et al. Pre-Hematopoietic stem cell transplant lung function and pulmonary complications in children. Annals of the American Thoracic Society. 2014; 11(10):1576-85. [DOI:10.1513/ AnnalsATS.201407-308OC] [PMID] [PMCID]
[19] Salvator H, Mahlaoui N, Catherinot E, Rivaud E, Pilmis B, Borie R, et al. Pulmonary manifestations in adult patients with chronic granulomatous disease. European Respiratory Journal. 2015; 45(6):1613-23. [DOI:10.1183/09031936.00118414] [PMID]
| Files | ||
| Issue | Vol 7 No 1 (2022): January-February | |
| Section | Case Report(s) | |
| DOI | https://doi.org/10.18502/crcp.v7i1.9628 | |
| Keywords | ||
| Bone marrow transplantation Chronic granulomatous disease Lung function Patient’s pulmonary function | ||
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How to Cite
1.
Rekabi M, Seif F, Nouri F, Mahdaviani A, Velayati AA. Two Successful Bone Functions in Patients with Chronic Granulomatous Disease Marrow Transplantations Improved Lung. CRCP. 2022;7(1):9-12.
