Volume 12, Issue 4 (10-2022)                   Iran J Ped Hematol Oncol 2022, 12(4): 272-302 | Back to browse issues page


XML Print


Department of Immunopathology, Institute of lungs Biology and Disease, Comprehensive Pneumology Center, Helmholtz Zentrum, 85764 Neuherberg, Munich, Germany
Abstract:   (703 Views)
Background: Sickle cell anemia (SCA) is an inherited monogenic disorder. The clinical symptoms of SCA are protean, including vaso-occlusion, hemolysis, early stroke, leg ulcers, multi-organ failure, and increased risk of premature death. Hematopoietic stem cell transplantation is the only treatment identified to reduce SCA-related organ damage. Unfortunately, graft rejection is a significant impediment to these strategies.
Materials and Methods: The current standard of treatment for the past two decades is limited to myeloablative-matched sibling donors, which is likely to be only for minor patients and is feasible for non-malignant giant disease. Cumulative studies showed that HSCT increases overall survival and quality of life in patients with SCA.
Results: Hematopoietic stem cell transplantation (HSCT) is significantly associated with a higher risk of graft versus host disease and moderate mortality risk. New strategy lacking standard donors includes cord blood, matched unrelated donors/ Haploidentical donors.
Conclusion: This review summarized evidence from HSCT clinical trials from different transplantation methods, specific HSCT and HSCT-related health problems that need to be addressed in medical contexts with patients and family members, and other areas that enhance the quality of life in SCA.
Full-Text [PDF 500 kb]   (640 Downloads)    
Type of Study: Research | Subject: General
Received: 2021/05/19 | Accepted: 2022/05/13 | Published: 2022/10/18

Rights and permissions
Creative Commons License This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.