Kasabach-Merritt Phenomenon: A Report of 11 Cases From a Single Institution

J Pediatr Hematol Oncol. 2013 Oct;35(7):554-8. doi: 10.1097/MPH.0b013e318281558e.

Abstract

Background: Kasabach-Merritt phenomenon (KMP) is a rare condition and optimal treatments have not yet been established, especially for cases that are unresponsive to first-line therapy. We retrospectively reviewed 11 KMP cases treated over the past 13 years in our institute.

Observations: With the exception of 1 case, steroids were administered as the first-line therapy. Eight cases required second-line or third-line therapy. The effective salvage therapies include interferon (n=1), radiotherapy (n=1), and chemotherapy (n=5). One case continues to depend upon chemotherapy. Three refractory cases were therapy dependent over 1 year of age, whereas 8 were treated effectively by 6 months of age.

Conclusions: Chemotherapy seems to be the most effective therapy for steroid-resistant KMP cases.

Publication types

  • Case Reports

MeSH terms

  • Combined Modality Therapy / adverse effects
  • Female
  • Humans
  • Infant
  • Infant, Newborn
  • Kasabach-Merritt Syndrome / blood
  • Kasabach-Merritt Syndrome / diagnosis*
  • Kasabach-Merritt Syndrome / therapy
  • Male
  • Retrospective Studies
  • Treatment Outcome