Improved T-Cell Chimerism and Successful Withdrawal of Immunosuppression after Non-Myeloablative Stem Cell Transplantation for Sickle Cell Disease with Azathioprine/Hydroxyurea Preconditioning Added to Alemtuzumab/TBI Conditioning

Dovern, Elisabeth; Aydin, Mesire; Tang, Man Wai; Suijk, Liesbeth E.M.; Hoogendoorn, Gerianne M.; Tuijn, Charlotte F.J. Van; Kerkhoffs, Jean-Louis; Rutten, Caroline E.; Hazenberg, Mette D.; Biemond, Bart J.; Nur, Erfan

doi:10.1182/blood-2022-159390

Cited by 1 publication

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Section: Methodsmentioning

confidence: 99%

“…Patients with a matched sibling donor underwent nonmyeloablative conditioning consisting of alemtuzumab and 3 Gy of total body irradiation (TBI), using peripheral blood stem cells. 24,25 Sirolimus was used as graft versus host disease (GvHD) and rejection prophylaxis during 1 year following HSCT. For patients undergoing nonmyeloablative haploidentical bone marrow transplantation, the conditioning consisted of antithymocyte globulin, fludarabine, cyclophosphamide, thiotepa, and TBI (2 Gy).…”

Section: Hsct Proceduresmentioning

confidence: 99%

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Cerebral hemodynamics and oxygenation in adult patients with sickle cell disease after stem cell transplantation

Afzali‐Hashemi,

Dovern,

Baas

et al. 2023

American J Hematol

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Sickle cell disease (SCD) is characterized by chronic hemolytic anemia associated with impaired cerebral hemodynamics and oxygen metabolism. Hematopoietic stem cell transplantation (HSCT) is currently the only curative treatment for patients with SCD. Whereas normalization of hemoglobin levels and hemolysis markers has been reported after HSCT, its effects on cerebral perfusion and oxygenation in adult SCD patients remain largely unexplored. This study investigated the effects of HSCT on cerebral blood flow (CBF), oxygen delivery, cerebrovascular reserve (CVR), oxygen extraction fraction (OEF), and cerebral metabolic rate of oxygen (CMRO2) in 17 adult SCD patients (mean age: 25.0 ± 8.0, 6 females) before and after HSCT and 10 healthy ethnicity‐matched controls (mean age: 28.0 ± 8.8, 6 females) using MRI. For the CVR assessment, perfusion scans were performed before and after acetazolamide as a vasodilatory stimulus. Following HSCT, gray and white matter (GM and WM) CBF decreased (p < .01), while GM and WM CVR increased (p < .01) compared with the baseline measures. OEF and CMRO2 also increased towards levels in healthy controls (p < .01). The normalization of cerebral perfusion and oxygen metabolism corresponded with a significant increase in hemoglobin levels and decreases in reticulocytes, total bilirubin, and LDH as markers of hemolysis (p < .01). This study shows that HSCT results in the normalization of cerebral perfusion and oxygen metabolism, even in adult patients with SCD. Future follow‐up MRI scans will determine whether the observed normalization of cerebral hemodynamics and oxygen metabolism prevents new silent cerebral infarcts.

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Section: Methodsmentioning

confidence: 99%

Section: Hsct Proceduresmentioning

confidence: 99%