Objective: Although the outcomes of treatment of Kasabach-Merritt syndrome (KMS) have improved over
time, some patients succumb to uncontrollable hemorrhage. Additionally, there is no consensus guideline
for the management of KMS. Clinical research on KMS might benefit physicians who treat such patients.
Methods: A retrospective chart review of patients diagnosed as KMS by Siriraj Hospital from 2006 to 2016 was conducted.
Results: Ten patients were diagnosed with KMS. Four patients underwent surgical intervention and obtained pathological
results; 3 of them had kaposiform hemangioendothelioma (KHE), while the fourth had infantile hemangioma (IH). The
combination of propranolol and prednisolone, with or without vincristine, was the most common first-line regimen,
with a complete response of 37.5%. A combination of vincristine, aspirin, and ticlopidine (VAT) was prescribed
as the second-line therapy for 5 patients, but there were no responses in this cohort. Another 2 patients attained
hematological remission with embolization and prednisolone monotherapy. A further 2 patients with KHE who were
refractory to other treatments responded well to sirolimus, while the tenth patient died of abdominal hemorrhage.
Conclusion: The combination of propranolol and prednisolone seems to be effective for KMS. Sirolimus may be
considered for salvage therapy for those whose disease is recalcitrant to standard treatment, especially in cases of
KMS secondary to KHE. However, research on a larger cohort should be conducted to substantiate the efficacy of
such treatments