Chronic transfusion therapy for children with sickle cell disease and recurrent acute chest syndrome

Jane Hankins, Michael Jeng, Sylvia Harris, Chin-Shang Li, Tiebin Liu, Winfred Wang

Research output: Contribution to journalArticle

38 Scopus citations

Abstract

The objective was to study the effects of chronic transfusion therapy (CTX) on the prevention of new episodes of acute chest syndrome (ACS) in children with sickle cell disease (SCD) and recurrent or unusually severe ACS. A retrospective chart review was performed of patients given CTX for recurrent or severe ACS. Frequency, median severity score, and median hospital stay for ACS episodes were determined. Differences in these values before and during CTX were analyzed. Twenty-seven patients were identified. Before treatment, the ACS incidence was 1.3 episodes per patient-year; during treatment, it decreased to 0.1 episodes per patient-year (P < 0.0001). The median severity score for ACS episodes was 0.8 (range 0-5) before CTX and 0.5 (0-3) during CTX (P = 0.84). The median hospital stay was 5 days (range 3-15 days) before CTX and 3 days (2-7 days) during CTX (P = 0.38). CTX significantly reduces the incidence of ACS events among patients with a history of recurrent or severe episodes but does not significantly decrease their severity. The effectiveness of CTX should be prospectively compared with that of hydroxyurea and stem cell transplantation.

Original languageEnglish (US)
Pages (from-to)158-161
Number of pages4
JournalJournal of Pediatric Hematology/Oncology
Volume27
Issue number3
DOIs
StatePublished - Mar 2005
Externally publishedYes

Keywords

  • Acute chest syndrome
  • Chronic transfusion
  • Iron overload
  • Pediatrics
  • Sickle cell disease

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Oncology
  • Hematology

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