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Kasabach-Merritt Syndrome: a case study of successful treatment with vincristine and propranolol

Aqsa Mazhar1, Afza Naureen Ghouse2, Saba Shahid3

  • 1Vascular Anomalies Center, Indus Hospital and Health Network, Karachi, Pakistan.

JPMA. the Journal of the Pakistan Medical Association
|December 12, 2023
PubMed

Insights

Kasabach-Merritt syndrome, a rare infant condition, involves vascular tumors and low platelets. Successful treatment with propranolol and vincristine was observed in a case report, highlighting a potential therapeutic approach.

Area of Science:

  • Pediatric Hematology Oncology
  • Vascular Anomalies
  • Rare Diseases

Background:

  • Kasabach-Merritt syndrome (KMS) is a rare, severe condition in infants.
  • It involves rapidly growing vascular tumors, thrombocytopenia, microangiopathic hemolytic anemia, and consumptive coagulopathy.
  • Current treatment guidelines for KMS are lacking, posing management challenges.

Observation:

  • A case report details a four-month-old female infant diagnosed with Kasabach-Merritt syndrome.
  • The infant presented with severe thrombocytopenia and a significant vascular tumor.
  • No prior standard treatment had been established for this specific presentation.

Findings:

  • The infant was treated with a combination of propranolol and vincristine.
  • This therapeutic regimen successfully reversed severe thrombocytopenia.
  • The treatment also led to a notable decrease in the size of the hemangioma.

Implications:

  • This case suggests propranolol and vincristine as a viable treatment option for Kasabach-Merritt syndrome.
  • Early diagnosis and prompt, monitored treatment are crucial for favorable outcomes in KMS.
  • Documenting individual case experiences is vital due to the rarity of KMS and the infeasibility of large-scale studies.
Keywords:
Kasabach-Merritt syndrome, Haemangioma, Thrombocytopenia.

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