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Prolongation of the prothrombin time and activated partial thromboplastin time in children with sickle cell disease

Leslie J Raffini1, Alison E Niebanck, Joanne Hrusovsky

  • 1Division of Hematology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104-4399, and Duke University School of Medicine, Durham, NC, USA. raffini@email.chop.edu

Pediatric Blood & Cancer
|August 27, 2005
PubMed

Insights

Children with sickle cell disease (SCD) often have prolonged prothrombin times (PTs) before surgery. Abnormal coagulation screening tests in SCD patients may indicate bleeding risks, warranting further investigation.

Area of Science:

  • Hematology
  • Pediatric Surgery
  • Coagulation Disorders

Background:

  • Sickle cell disease (SCD) is associated with increased perioperative complications, particularly bleeding.
  • Understanding coagulation abnormalities in pediatric SCD patients is crucial for surgical risk assessment.

Purpose of the Study:

  • To determine the prevalence of abnormal coagulation screening tests in pediatric patients with SCD.
  • To evaluate potential etiologies for these coagulation abnormalities.
  • To assess the association between prolonged PTs and perioperative bleeding in SCD.

Main Methods:

  • Retrospective review of pre-operative coagulation studies in pediatric SCD patients.
  • Prospective study of 100 well children with SCD to assess coagulation parameters.
  • Analysis of prothrombin time (PT) and activated partial thromboplastin time (aPTT) results.
  • Evaluation of coagulation factor levels (Factor V, VII) and vitamin K status.

Main Results:

  • 38.1% of retrospective SCD patients had prolonged PTs; 8% in the prospective study.
  • Prolonged PTs in prospective SCD patients correlated with lower Factor V and VII levels.
  • Factor VII levels were significantly lower in SCD patients with prolonged PTs (P=0.001).
  • Six of seven SCD children with prolonged PTs experienced significant intraoperative blood loss (P=0.04).

Conclusions:

  • Pediatric SCD patients undergoing surgery are more likely to exhibit prolonged PTs compared to healthy controls.
  • Coagulation abnormalities in SCD may stem from clinical status, liver dysfunction, or factor consumption.
  • Further prospective studies are needed to confirm the link between abnormal coagulation tests and perioperative bleeding risk in pediatric SCD.
Abstract

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