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Splenic Complications in Pediatric Sickle Cell Disease: A Retrospective Cohort Review
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  • Alex George,
  • Shannon E. Conneely,
  • Ross Mangum,
  • Titilope Fasipe,
  • Philip Lupo,
  • Michael Scheurer
Alex George
Wake Forest University School of Medicine

Corresponding Author:[email protected]

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Shannon E. Conneely
Baylor College of Medicine Department of Pediatrics
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Ross Mangum
Phoenix Children's Hospital Center for Cancer and Blood Disorders
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Titilope Fasipe
Baylor College of Medicine Department of Pediatrics
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Philip Lupo
Baylor College of Medicine Department of Pediatrics
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Michael Scheurer
Baylor College of Medicine Department of Pediatrics
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Abstract

Objective: To delineate the natural history of splenic complications other than the loss of splenic function in children with sickle cell disease (SCD), we performed a retrospective chart review of patients with SCD treated at the Texas Children’s Hospital. Methods: We determined the dates of diagnoses of splenic complications, the number of ASSC events, and hydroxyurea treatment in patients with SCD. We also examined the association of hydroxyurea therapy with the onset and severity of ASSC. Results: The cumulative prevalence of splenic complications was 24.7% for splenomegaly, 24.2% for ASSC, 9.6% for hypersplenism, and 5.6% for splenectomy. The cumulative prevalence of all splenic complications was highest in patients with hemoglobin Sβ 0 (69.2%), intermediate in hemoglobin SS (33.3%), low in hemoglobin SC (9.0%), and non-existent in hemoglobin Sβ +. The overall event-rate of ASSC was 8.3 per hundred patient-years. The event-rate was 28.4 in the hemoglobin Sβ 0, 10.9 in hemoglobin SS, and 3.5 in hemoglobin SC Patients with hemoglobin SS and hemoglobin Sβ 0 on hydroxyurea therapy had a significantly higher occurrence of ASSC than those who were not, with event-rates of 14.2 and 3.1, respectively. The event-rate was also higher for children who started hydroxyurea before age 2 years than for those who started after this age (19.8 and 9.2 respectively). Conclusions: The prevalence and severity of splenic problems vary widely between different sickle cell genotypes, with hemoglobin Sβ 0 having the most severe complications. Hydroxyurea therapy is strongly associated with incidence of ASSC, particularly when initiated before two years of age.
Submitted to Pediatric Blood & Cancer
09 Mar 2024Submission Checks Completed
09 Mar 2024Assigned to Editor
11 Mar 2024Review(s) Completed, Editorial Evaluation Pending
27 Mar 2024Editorial Decision: Revise Major
15 Jun 2024Submission Checks Completed
15 Jun 2024Assigned to Editor
15 Jun 20242nd Revision Received
17 Jun 2024Reviewer(s) Assigned
03 Jul 2024Review(s) Completed, Editorial Evaluation Pending