Coexisting or underlying risk factors of hepatic veno-occlusive disease in pediatric hematopoietic stem cell transplant recipients receiving prophylaxis

Exp Clin Transplant. 2013 Oct;11(5):440-6. doi: 10.6002/ect.2012.0265.

Abstract

Objectives: To evaluate the characteristics of veno-occlusive disease in pediatric hematopoietic stem cell transplant recipients, and their effect as a prophylactic regimen on severity and outcome.

Materials and methods: This study included 204 allogeneic hematopoietic stem cell transplants performed on 187 children whose data retrospectively described the risk factors, prophylaxis, and treatment modalities of veno-occlusive disease. A prophylactic regimen composed of enoxaparin versus ursodeoxycholic acid and vitamin E was given to 167 of 204 patients.

Results: Veno-occlusive disease developed in 22 patients (10.8%). Nineteen patients experienced veno-occlusive disease despite this prophylactic regimen. The prophylaxis seemed ineffective in preventing veno-occlusive disease (P = .657). Regarding risk factors, oral busulphan use, liposomal amphotericin B vancomycin treatment, and total parenteral nutrition were associated with an increased risk of veno-occlusive disease. Conversely, renal impairment also was associated with increased mortality in patients with veno-occlusive disease. The mortality rate in the first 100 days after a hematopoietic stem cell transplant was higher in the patients with veno-occlusive disease than it was in those without the disease.

Conclusions: Our prophylactic regimen may have played a role in the fairly low incidence of veno-occlusive disease in a pediatric population with high-risk features.

MeSH terms

  • Administration, Oral
  • Adolescent
  • Age Factors
  • Amphotericin B / adverse effects
  • Anticoagulants / therapeutic use
  • Antifungal Agents / adverse effects
  • Busulfan / administration & dosage
  • Busulfan / adverse effects
  • Child
  • Child, Preschool
  • Drug Therapy, Combination
  • Enoxaparin / therapeutic use
  • Female
  • Hematopoietic Stem Cell Transplantation / adverse effects*
  • Hematopoietic Stem Cell Transplantation / mortality
  • Hepatic Veno-Occlusive Disease / diagnosis
  • Hepatic Veno-Occlusive Disease / epidemiology*
  • Hepatic Veno-Occlusive Disease / mortality
  • Hepatic Veno-Occlusive Disease / prevention & control
  • Humans
  • Immunosuppressive Agents / administration & dosage
  • Immunosuppressive Agents / adverse effects
  • Incidence
  • Infant
  • Kaplan-Meier Estimate
  • Kidney Diseases / epidemiology
  • Male
  • Parenteral Nutrition, Total / adverse effects
  • Proportional Hazards Models
  • Retrospective Studies
  • Risk Factors
  • Time Factors
  • Treatment Outcome
  • Turkey
  • Ursodeoxycholic Acid / therapeutic use
  • Vitamin E / therapeutic use
  • Young Adult

Substances

  • Anticoagulants
  • Antifungal Agents
  • Enoxaparin
  • Immunosuppressive Agents
  • liposomal amphotericin B
  • Vitamin E
  • Ursodeoxycholic Acid
  • Amphotericin B
  • Busulfan