TY - JOUR AU - Buaboonnam, Jassada AU - Sanpakit, Kleebsabai AU - Narkbunnam, Nattee AU - Vathana, Nassawee AU - Sowithayasakul, Panjarat AU - Anuntarumporn, Lillada AU - Nitiyarom, Rattanavalai PY - 2017/12/20 Y2 - 2024/03/29 TI - Treatment Outcomes of Kasabach-Merritt Syndrome: A Single Hospital Experience JF - Siriraj Medical Journal JA - Siriraj Med J VL - 69 IS - 6 SE - Original Article DO - UR - https://he02.tci-thaijo.org/index.php/sirirajmedj/article/view/106176 SP - 351-355 AB - <p><strong>Objective:</strong> Although the outcomes of treatment of Kasabach-Merritt syndrome (KMS) have improved over&nbsp;time, some patients succumb to uncontrollable hemorrhage. Additionally, there is no consensus guideline&nbsp;for the management of KMS. Clinical research on KMS might benefit physicians who treat such patients.<br><strong>Methods:</strong> A retrospective chart review of patients diagnosed as KMS by Siriraj Hospital from 2006 to 2016 was conducted.&nbsp;Results: Ten patients were diagnosed with KMS. Four patients underwent surgical intervention and obtained pathological&nbsp;results; 3 of them had kaposiform hemangioendothelioma (KHE), while the fourth had infantile hemangioma (IH). The&nbsp;combination of propranolol and prednisolone, with or without vincristine, was the most common first-line regimen,&nbsp;with a complete response of 37.5%. A combination of vincristine, aspirin and ticlopidine (VAT) was prescribed&nbsp;as the second-line therapy for 5 patients, but there were no responses in this cohort. Another 2 patients attained&nbsp;hematological remission with embolization and prednisolone monotherapy. A further 2 patients with KHE who were&nbsp;refractory to other treatments responded well to sirolimus, while the tenth patient died of abdominal hemorrhage.<br><strong>Conclusion:</strong> The combination of propranolol and prednisolone seems to be effective for KMS. Sirolimus may be&nbsp;considered for salvage therapy for those whose disease is recalcitrant to standard treatment, especially in cases of&nbsp;KMS secondary to KHE. However, research on a larger cohort should be conducted to substantiate the efficacy of&nbsp;such treatments.</p> ER -