Contribution of fibrinolytic tests to the differential diagnosis of veno-occlusive disease complicating pediatric hematopoietic stem cell transplantation

Pediatr Blood Cancer. 2012 May;58(5):791-7. doi: 10.1002/pbc.23213. Epub 2011 Jun 14.

Abstract

Background: Veno-occlusive disease (VOD) is a major complication following hematopoietic stem cell transplantation (HSCT). Its diagnosis is based on clinical criteria, which have a limited sensitivity. Increased plasminogen activator inhibitor-1 (PAI-1) levels have been suggested as a marker of VOD. We aimed to prospectively evaluate how the fibrinolytic parameters behaved to discriminate VOD from other liver disorders occurring after HSCT in a pediatric population.

Procedures: A total of 195 HSCT were performed on 161 children and VOD complicated 11 cases (6.8%). Alanine aminotransferase, total bilirubin, PAI-1 antigen (PAI-1:Ag) and activity, t-PA antigen, D-dimer, prothrombin time, activated partial thromboplastin time, antithrombin, fibrinogen, and platelet counts were measured in 105 HSCT before and then weekly for 1 month after HSCT.

Results: An early, significant increase in the fibrinolytic parameters was seen in patients who developed VOD, even before VOD was diagnosed clinically, by comparison with patients without complications or those with non-VOD liver disorders. The combined increase in bilirubin, D-dimer, and PAI-1:Ag levels beyond the normal range distinguished VOD cases from other liver complications with a high sensitivity and specificity.

Conclusions: Our study demonstrates that fibrinolytic tests can help diagnose VOD after HSCT in the pediatric population.

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Diagnosis, Differential
  • Female
  • Fibrinolysis*
  • Hematopoietic Stem Cell Transplantation / adverse effects*
  • Hepatic Veno-Occlusive Disease / diagnosis*
  • Hepatic Veno-Occlusive Disease / etiology
  • Humans
  • Infant
  • Male
  • Prospective Studies
  • Sensitivity and Specificity