AI Article Synopsis

  • - Kasabach-Merritt phenomenon (KMP) is a rare condition with unclear optimal treatments, particularly for those unresponsive to standard therapies; 11 cases from a 13-year review were analyzed.
  • - Most patients received steroids as their initial treatment, but eight needed additional therapies, including interferon, radiotherapy, and chemotherapy, with chemotherapy showing notable effectiveness.
  • - The findings suggest that chemotherapy is particularly beneficial for cases of KMP that do not respond to steroids, especially in patients older than one year who remained dependent on therapy.

Article 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.

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Source
http://dx.doi.org/10.1097/MPH.0b013e318281558eDOI Listing

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