关键词: Kasabach-Merritt综合征, 治疗结果, 手术疗法

Abstract: 【Abstract】 Objective To analyze efficacy of comprehensive surgical treatment for Kasabach-Merritt phenomenon（KMP）. Methods Clinical data were collected from 18 patients with KMP who underwent surgical treatment in Department of Hemangioma, Henan Provincial People′s Hospital from November 2017 to March 2019, and analyzed retrospectively. Preoperative treatment included preoperative use of glucocorticoids and immunoglobulins to increase platelet counts in patients. For patients irresponsive to glucocorticoids, a single large-dose of platelets was infused 12 - 24 hours before surgery. The platelet dose for infusion should be 0.3 and 0.2 therapeutic dose （TD）/kg respectively for patients with platelet counts lower than 30 × 109/L and those with platelet counts higher than 30 × 109/L, and the total infusion dose should be no more than 1 TD （containing about 2.5 × 1011 platelets in 200 - 250 ml of infusion solution）. During the operation, tumor tissues were removed as much as possible; if there were difficulties in suturing in patients with large tumors, in situ autologous skin grafting would be performed; for children with potential postoperative functional dysfunction, a clinical consultation with rehabilitation specialists was needed, and individualized functional exercises should be given to promote functional restoration. Results Eighteen patients were enrolled, including 9 males and 9 females, with an average age of 73 days （range, 7 - 354 days）. Skin lesions were located on the extremities in 3 cases, on the trunk in 11, and on the head, face and neck in 4. Surgeries were successful in 17 patients, but 1 died. Among the 17 patients with successful operation, platelet counts and coagulation function returned to normal within 1 week after the surgery in 16, and did not returned to normal until after regular oral administration of sirolimus in 1. Favorable movement ability was maintained in all the children. Conclusion Comprehensive surgical treatment shows rapid efficacy with less adverse reactions in the therapy of KMP.

Key words: Kasabach-Merritt syndrome, Treatment outcome, Surgical therapy