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Lower Gastrointestinal Bleeding Because of Kasabach-Merritt Syndrome Showing an Impressive Response to Sirolimus

Jorge Sandelis Pérez1, Dahima Cintrón2, Alexandra Deyá-Quinquilla3

  • 1Internal Medicine Department, University of Puerto Rico-Medical Sciences Campus, San Juan, Puerto Rico.

ACG Case Reports Journal
|June 23, 2022
PubMed

Insights

Kasabach-Merritt syndrome, a rare vascular tumor complication, typically affects children. This case highlights successful sirolimus treatment in an adult with severe gastrointestinal bleeding from this condition.

Area of Science:

  • Vascular biology
  • Pediatric oncology
  • Hematology

Background:

  • Kasabach-Merritt syndrome (KMS) is a rare, life-threatening condition characterized by vascular tumors causing coagulopathy.
  • KMS typically presents in infants and young children with complications like thrombocytopenia and hemolytic anemia.

Observation:

  • This report details an adult patient experiencing recurrent, severe lower gastrointestinal bleeding.
  • The bleeding was attributed to Kasabach-Merritt syndrome, a presentation uncommon in adults.

Findings:

  • The adult patient with KMS and severe gastrointestinal bleeding was treated with sirolimus.
  • Sirolimus therapy proved successful in managing the bleeding and associated coagulopathy.

Implications:

  • This case expands the known clinical spectrum of Kasabach-Merritt syndrome to include adult presentations.
  • Sirolimus represents a potential therapeutic option for adult KMS patients, particularly those with refractory bleeding.

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