P118: The Role of Hemoglobin and Hemolysis on Transcranial Doppler Velocities in Children With Sickle Cell Disease: Data From a Natural History Cohort

Reggiani, Giulia; Coppadoro, Beatrice; Munaretto, Vania; Manara, Renzo; Baracchini, Claudio; Pieroni, Alessio; Viaro, Federica; Agodoa, Irene; Beaubrun, Anne; Biffi, Alessandra; Sainati, Laura; Colombatti, Raffaella

doi:10.1097/01.hs9.0000821564.69437.ee

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“…Surrogates of cerebral function, such as cerebral hemodynamics and/or vasculopathy measured by MRI, transcranial Doppler or MRA, improved after HSCT. The decreases in cerebral blood flow and maximal transcranial Doppler velocities might (at least partly) result from the normalization of hemoglobin after HSCT, as both parameters have been shown to strongly correlate with hemoglobin levels 62–64 . Previous studies found markers of impaired hemodynamics, such as cerebral blood flow and silent cerebral infarcts to be associated with deficits in cognitive function domains in children with SCD 65–67 .…”

Section: Discussionmentioning

confidence: 98%

“…The decreases in cerebral blood flow and maximal transcranial Doppler velocities might (at least partly) result from the normalization of hemoglobin after HSCT, as both parameters have been shown to strongly correlate with hemoglobin levels. [62][63][64] Previous studies found markers of impaired hemodynamics, such as cerebral blood flow and silent cerebral infarcts to be associated with deficits in cognitive function domains in children with SCD. [65][66][67] Further, silent infarcts were identified as risk factor for overt stroke.…”

Section: Discussionmentioning

confidence: 99%

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Effect of allogeneic hematopoietic stem cell transplantation on sickle cell disease‐related organ complications: A systematic review and meta‐analysis

Dovern,

Aydin,

DeBaun

et al. 2024

American J Hematol

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Sickle cell disease (SCD)‐related organ complications are a major cause of morbidity and mortality in patients with SCD. We sought to assess whether hematopoietic stem cell transplantation (HSCT) stabilizes, attenuates, or exacerbates organ decline. We performed a systematic review and meta‐analysis of trials investigating organ function before and after HSCT in patients with SCD. We searched MEDLINE/PubMed and EMBASE up to September 21, 2023. Continuous data were expressed as standardized mean difference (SMD) and pooled in a weighted inverse‐variance random‐effects model; binomial data were expressed as risk ratio (RR) using the Mantel–Haenszel random‐effects meta‐analyses. Of 823 screened studies, 34 were included in this review. Of these, 17 (774 patients, 23.6% adults, 86.3% HLA‐identical sibling donor, 56.7% myeloablative conditioning regimen) were included in the meta‐analyses. Pulmonary function remained stable. Mean tricuspid regurgitant jet velocity decreased but did not reach statistical significance. In children, estimated glomerular filtration rate decreased (SMD −0.80, p = .01), and the presence of proteinuria increased (RR 2.00, p = <.01), while splenic uptake and phagocytic function improved (RR 0.31, p = <.01; RR 0.23, p = <.01). Cerebral blood flow improved (SMD −1.39, p = <.01), and a low incidence of stroke after transplantation in high‐risk patients was found. Retinopathy and avascular osteonecrosis were investigated in only one study, showing no significant changes. While HSCT can improve some SCD‐related organ dysfunctions, transplantation‐related toxicity may have an adverse effect on others. Future research should focus on identifying individuals with SCD who might benefit most from HSCT and which forms of organ damage are more likely to exacerbate post‐transplantation.

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Section: Discussionmentioning

confidence: 98%