Is the Game Over or Starting Again? The Role of the Transplant Team in Genetic Counseling for Adult Sickle Cell Disease Recipients

Hematopoietic stem cell transplantation (HSCT) with nonmyeloablative regimens has become more feasible and it is currently performed more frequently in adult patients with sickle cell disease (SCD) [1]. Hsieh [1] reported successful outcomes for adults with a regimen of alemtuzumab and low-dose total body irradiation (TBI) in transplants from human leukocyte antigen (HLA)-matched sibling donors. We have obtained sustained full chimerism in 13 adults with protocols including antithymocyte globulin (ATG) and busulfan without any significant complications (unpublished data). For unrelated donors, the ongoing STRIDE study uses conditioning regimens containing ATG, fludarabine, and busulfan. Bolaños-Meade et al. [2] achieved a cure with HLA-haploidentical transplants performed with ATG, fludarabine, and low-dose TBI. Given that these protocols may allow assisted or spontaneous pregnancies, fertility issues need to be examined [3] because the patient may think that he/she is completely cured after allogeneic transplantation and that he/she no longer carries the hemoglobin S (Hb S) gene. This erroneous belief may lead to the delivery of a homozygous Hb SS infant if the partner has a high risk of being a Hb S gene carrier when marriages of close relatives occur, as in some populations like Eti-Turks.