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Prolongation of the prothrombin time and activated partial thromboplastin time in children with sickle cell disease
Author(s) -
Raffini Leslie J.,
Niebanck Alison E.,
Hrusovsky Joanne,
Stevens Amanda,
BlackwoodChirchir Anne,
OheneFrempong Kwaku,
Kwiatkowski Janet L.
Publication year - 2005
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.20579
Subject(s) - medicine , partial thromboplastin time , prospective cohort study , prothrombin time , perioperative , retrospective cohort study , coagulation testing , coagulation , coagulation disorder , gastroenterology , etiology , clotting factor , surgery
Background Patients with sickle cell disease (SCD) have high rates of perioperative complications, including bleeding 1,2. Procedures We conducted a retrospective review of pre‐operative coagulation studies in pediatric patients with SCD followed by a prospective study of 100 well children with SCD to determine the prevalence of abnormal coagulation screening tests, and to evaluate potential etiologies. Results In the retrospective study, 32/84 (38.1%) had a prolonged prothrombin time (PT), compared to 8/100 in the prospective study. Prolongations of the activated partial thromboplastin time (aPTT) were less common. Children in the prospective study with prolonged PTs had significantly lower levels of Factor V and VII compared to those with normal PTs. Factor VII levels were <50% in 4/8 with long PTs, compared to 3/92 with normal PTs, P = 0.001. Though retrospectively, several patients had normalization of their PT with vitamin K, there was no laboratory evidence of vitamin K deficiency in the prospective study. In the retrospective analysis, six of seven children who had pre‐operative coagulation studies and significant intraoperative blood loss had prolonged PTs ( P = 0.04). Conclusions Children with SCD admitted for surgical procedures were more likely to have prolonged PTs than those tested at a well visit. There was intra‐patient variability in coagulation studies that may be related to clinical status, hepatocellular dysfunction, and/or increased clotting factor consumption. Future well‐designed prospective studies to determine whether abnormal coagulation studies are associated with an increased risk of perioperative bleeding in children with SCD are necessary. Pediatr Blood Cancer 2006; 47:589–593. © 2005 Wiley‐Liss, Inc.