[Combined and sequential therapy for Kasabach-Merritt syndrome].

Objective: To review and summarize the clinical characteristics and therapeutic approaches of Kasabach-Merritt syndrome (KMS).

Methods: The combined and sequential therapy was used to for 51 KMS infants between 2005 and 2009. And all cases were retrospectively analyzed. There were 27 males and 24 females with a median age of 1.8 months (range: 2 days to 2.5 years). Among them, lesions were located in maxillofacial (n = 20), truck (n = 4), upper extremities (n = 11) and lower extremities (n = 16).

Results: Among them, 47 of 51 patients were cured, 3 cases were effectively treated and only 1 patient died of multiple organ failure post-operation. Of the 51 cases, emergency surgery was given in 3 cases while other 48 cases were initially treated with methylprednisolone. The responses were varied: excellent and rapid improvement (n = 12); moderate response (n = 15) and total failure (n = 21). Immunoglobulin was used a second-line therapy for those (n = 21) unresponsive to steroid, with sensitive response (n = 5), effective response (n = 7) and invalid response (n = 9) respectively. And adjunct carbonyldiamide therapy was combined. Conservative treatment was tried in 14 patients. Then 11 curative cases and 3 effective cases were observed. The thrombocyte count in 4 cases returned to normal within 4 - 7 days, 5 cases within 8 - 14 days, and 2 cases within 15 - 28 days. In the remaining 3 cases, it normalized more than 28 days. The hemangiomas in 6 cases disappeared completely in 6 - 12 months and 8 cases disappeared in 13 - 24 months. 19 patients were treated with the ligation of external carotid artery, the insertion of a tube inside the lesion and the injections of carbonyldiamide and methylprednisolone. The platelet counts of 13 cases increased to normal in 4 - 7 days of the treatment, and 6 cases in 8 - 14 days. The tumors of 12 cases disappeared within 6 - 12 months, and 7 cases within 13 - 24 months. Combined surgical resection including emergency operation (n = 3) and selective operation (n = 15) was performed in 18 patients. And all cases except for one were cured. The platelet counts of that the hemangiomas of 10 cases were excised surgically completely increased rapidly and reached the normal range in post-operative 1 - 3 days. The counts of 8 cases after subtotal resection increased to normal within 8-14 days.

Conclusion: A stepwise multimodal approach is recommended for the treatment of KMS. With a higher curative rate, it has minimal side effects.