Treatment Outcomes of Kasabach-Merritt Syndrome: A Single Hospital Experience

Authors

  • Jassada Buaboonnam Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok 10700
  • Kleebsabai Sanpakit Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok 1
  • Nattee Narkbunnam Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok 10700
  • Nassawee Vathana Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok 10700
  • Panjarat Sowithayasakul Department of Pediatrics, Faculty of Medicine, Srinakharinwirot University, Bangkok 10110
  • Lillada Anuntarumporn Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok 10700
  • Rattanavalai Nitiyarom Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok 10700

Keywords:

Kaposiform hemangioendothelioma; Kasabach-Merritt syndrome; prednisolone; propranolol; sirolimus

Abstract

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.

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Published

20-12-2017

How to Cite

Buaboonnam, J., Sanpakit, K., Narkbunnam, N., Vathana, N., Sowithayasakul, P., Anuntarumporn, L., & Nitiyarom, R. (2017). Treatment Outcomes of Kasabach-Merritt Syndrome: A Single Hospital Experience. Siriraj Medical Journal, 69(6), 351–355. Retrieved from https://he02.tci-thaijo.org/index.php/sirirajmedj/article/view/106176

Issue

Vol. 69 No. 6 (2017): November-December

Section

Original Article

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