Successful treatment of kasabach-merritt syndrome with vindesine: a case report and review of literature

Kasabach-Merritt Syndrome (KMS) is associated with profound thrombocytopenia and consumptive co-agulopathy caused by vascular tumors. In this report, we described an adult patient with KMS who received chemotherapy including vindesine (VDS), and briefly reviewed the literature on KMS treatment. This female patient was found to be presented with multiple subcutaneous masses at birth. These soft masses increased in size and numbers every year and led to KMS finally, including subarachnoid hemorrhage and coagulopathy. After being treated with combination of glucocorticoid, thalidomide and propranolol, her symptoms did not improve. We started VDS treatment on a 4 mg weekly basis for the first week, and then reduced to 2 mg weekly. After 4 weeks treatment, the platelets count and fibrinogen level both increased and no more newly emerged abnormal lesions was found. Based on these existing literatures, the current major treatments of KMS include physical therapy, traditional chemotherapy and hemostatic therapy. The treatment choice depends on the severity of KMS. For those patients who got widely distributed hemangiomas, and involving deep muscles and many important organs, will lose the opportunity of surgery excision or embolization treatment. The VDS based combination treatment approach may make the disease reached a stable state.