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. 2024 Dec;64(12):2260-2269.
doi: 10.1111/trf.18041. Epub 2024 Nov 5.

Longitudinal outcomes of chronically transfused adults with sickle cell disease and a history of childhood stroke

Jennifer M Jones  1 , Julia Wool  2 , Elizabeth P Crowe  3 , Evan M Bloch  3 , Lydia H Pecker  2 , Sophie Lanzkron  4
Affiliations

Longitudinal outcomes of chronically transfused adults with sickle cell disease and a history of childhood stroke

Jennifer M Jones et al. Transfusion. 2024 Dec.

Abstract

Background: Many children with sickle cell disease (SCD) who suffer a stroke receive chronic transfusion therapy (CTT) indefinitely; however, their adulthood neurologic outcomes have not been reported. Understanding these outcomes is critical to inform decisions regarding curative therapy in childhood.

Study design and methods: In this retrospective study, we described a cohort of adults with SCD and a history of childhood stroke who received care at a single center and compared their outcomes with matched subjects without childhood stroke using chi2 and Mann-Whitney U tests.

Results: Of 42 subjects with childhood stroke, all received CTT for secondary stroke prophylaxis. Five (11%) developed recurrent stroke. The rate of stroke was similar in subjects with and without childhood stroke (0.7 vs. 1.1 per 100 person·years, p = .63). Both cohorts exhibited evidence of iron overload (median ferritin 2227 vs. 1409 ng/dL, p = .10) and alloimmunization (45% vs. 45%, p = 1.0), despite receiving care in a comprehensive SCD program.

Discussion: For adults with SCD who had a childhood stroke, our results suggest CTT returns the risk of stroke to that of age-matched stroke naïve patients with SCD.

Keywords: cerebrovascular accident; chronic red cell exchange transfusions; sickle cell disease; therapeutic apheresis.

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Conflict of interest statement

The authors have disclosed no conflicts of interest.

Figures

FIGURE 1
FIGURE 1
Prevalence of sickle cell disease (SCD) comorbidities in subjects with and without a history of childhood stroke. The prevalence of avascular necrosis (4 vs. 14, p = .01) and leg ulcers (0 vs. 6, p = .02) was lower in subjects with a history of childhood stroke as compared to subjects without. The prevalence of chronic kidney disease (Stage I or greater) trended lower in subjects with a history of childhood stroke (14 vs. 21, p = .13). Differences in the prevalence of other SCD comorbidities (retinopathy, acute chest syndrome, priapism, chronic kidney disease, elevated tricuspid jet velocity [TRJV]) between the cohorts were not observed.
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