Hulbert ML, Fields ME, Guilliams KP, et al. Normalization of Cerebral Hemodynamics Following Hematopoietic Stem Cell Transplant in Children with Sickle Cell Disease. Blood. 2022; (doi: 10.1182/blood.2022016618).

Researchers report the normalization of cerebral hemodynamics in children with sickle cell disease (SCD) following hematopoietic stem cell transplant (HSCT). The authors used magnetic resonance imaging to quantify whole-brain cerebral blood flow (CBF) and oxygen extraction fraction (OEF) 1-3 months before and 12-24 months after HSCT in 10 children with SCD. Three children suffered prior overt strokes, five had prior silent strokes, and one exhibited abnormal transcranial Doppler ultrasound velocities. The children's CBF and OEF were compared with those of non-SCD controls and SCD participants who received chronic red blood cell transfusion therapy (CRTT) before and after a scheduled transfusion. Among the 10 children, seven underwent matched sibling donor HSCT and three had 8/8 matched unrelated donor HSCT. The children all received reduced-intensity preparation, and they were able to maintain engraftment without hemolytic anemia or SCD symptoms. Compared with the non-SCD controls, elevated CBF and OEF were observed before transplant, but those levels dropped substantially 1-2 years following HSCT. CBF and OEF after HSCT resembled that of non-SCD controls, and the post-HSCT CBF and OEF reduction was greater in transplant recipients than in SCD patients undergoing CRTT after a scheduled transfusion. Researchers conclude that this normalization of cerebral hemodynamics after HSCT in children with SCD leads to restoration of cerebral oxygen reserve which may explain stroke protection following HSCT in this SCD population.

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Tags: patient care, children, Cell, Cellular therapy, HSCT, hematopoietic, unrelated donor, sickle cell disease, hematopoietic cell transplantation