Purpose: To explore the diagnosis and treatment of hemangioma and vascular malformation associated with thrombopenia (Kasabach-Merritt syndrome, KMS).
Methods: From October 1997 to December 2003, 13 cases of KMS were treated in our hospital. Among the 13 patients, 4 were located in the maxillofacial region, 3 were located in the trunk, 6 were located in the lower limb. The size of the lesion of all patients exceeded 8 cm; 10 were hemangioma, 1 was arteriovenous malformation (AVM), 1 was venous malformation (VM), 1 was Klippel-Trenaunay Syndrome (KTS). The platelet count was all lower than 70 x 10(9)/L, the lowest was 10 x 10(9)/L, the average was 41 x 10(9)/L. The clinical characteristics and course of treatment were analyzed.
Results: 9 patients were cured, 1 improved, 1 had no response, and 2 died. The treatment of choice for KMS was steroids, but the response rate was not high (23.08% in this series). If patients had no response to steroids, they also had no response to interferon.
Conclusions: If a proper treatment was taken in early stage, most patients could get a good result, but for patients with an extensive vascular malformation that can't be removed, the prognosis was poor. For lesions in the limbs and trunk, pneumatic compression therapy has certain curative effect, fewer side effects, therefore worthy of popularization.