Ethical issues of unrelated hematopoietic stem cell transplantation in adult thalassemia patients

Abstract

BackgroundBeta thalassemia major is a severe inherited form of hemolytic anemia that results from ineffective erythropoiesis. Allogenic hematopoietic stem cell transplantation (HSCT) remains the only potentially curative therapy. Unfortunately, the subgroup of adult thalassemia patients with hepatomegaly, portal fibrosis and a history of irregular iron chelation have an elevated risk for transplantation-related mortality that is currently estimated to be about 29 percent.DiscussionThalassemia patients may be faced with a difficult choice: they can either continue conventional transfusion and iron chelation therapy or accept the high mortality risk of HSCT in the hope of obtaining complete recovery.Throughout the decision making process, every effort should be made to sustain and enhance autonomous choice. The concept of conscious consent becomes particularly important. The patient must be made fully aware of the favourable and adverse outcomes of HSCT. Although it is the physician's duty to illustrate the possibility of completely restoring health, considerable emphasis should be put on the adverse effects of the procedure. The physician also needs to decide whether the patient is eligible for HSCT according to the "rule of descending order". The patient must be given full details on self-care and fundamental lifestyle changes and be fully aware that he/she will be partly responsible for the outcome.SummaryOnly if all the aforesaid conditions are satisfied can it be considered reasonable to propose unrelated HSCT as a potential cure for high risk thalassemia patients.

A Child as a Donor for Hematopoietic Stem Cell Transplantation: Bioethical Justification—A Case Study on Sickle Cell Disease

“…Although studies have shown that iron overload caused by regular transfusions can be controlled by an oral iron chelator [ 34 ], our patient had already have severe blood transfusion consequences. Furthermore, these problems could more probably reduce survival and quality of life than the HSCT procedure itself.…”

“…Furthermore, these problems could more probably reduce survival and quality of life than the HSCT procedure itself. Therefore, the beneficence and the nonmaleficence principles were respected in this point of view [ 34 ].…”

“…An important factor to consider in such cases is also the competent support from relatives and/or caregivers during the pre- and post-HSCT period [ 34 ]. In our case, these parents and/or caregivers would be able to support the donor and the recipient (their daughter and son).…”

“…Finally, our patient, his donor, and their parents were able to comprehend the procedure, and they had the educational, cultural, family, and social background according to the “rule of descending order.” This rule suggests that patients have less knowledge than the physician, justifying our efforts to inform them adequately [ 34 ].…”

β-thalassemia caused by compound heterozygous mutations and cured by bone marrow transplantation: A case report

“…Currently, hematopoietic stem cell transplantation (HSCT) is the only effective way to cure severe β-thalassemia in the clinical practice ( 15 , 26 , 27 ). The influence on transplant success rate of donor mainly depends on the HLA typing on chromosome 6 ( 28 ).…”