Kasabach-Merritt phenomenon (KMP) is life-threatening, charactered by the profound
thrombocytopenia and consumptive coagulopathy associated with vascular
tumors. The
therapy of KMP still remains challenging. In this study, we retrospectively analyzed the clinical data of KMP treated in Nanjing Children's Hospital and Jinling Hospital, China, and brief reviewed the literature on KMP. From Jan. 2005 to Dec. 2014, a total of 19 cases of KMP were enrolled into this study. Laboratory results showed that seven patients had typical
disseminated intravascular coagulation (
DIC), and others were atypical
DIC. CT scanning showed the low-density
tumor with obvious intensification in enhanced scanning, and the large distorted arteries in association with the
tumor. After the admission, the patients received the infusion of platelets and the applying of
dipyridamole,
steroids, and other necessary drugs. Eight patients underwent complete surgical removal of the
tumor, or partial removal with subsequent
chemotherapy of
vincristine. Three patients underwent only the
chemotherapy of
vincristine. Eight patients underwent the
intralesional injection of absolute
ethanol. Pathological examination showed eighteen samples were
kaposiform hemangioendothelioma, and one tufted agioma. In our cases, six patients died from extensive
hemorrhage and subsequent
multiple organ failure. The others survived. In conclusion, KMP in Chinese children has typical symptoms.
Kaposiform hemangioendothelioma is the most frequent vascular
tumor associated with KMP. The individual treatments with surgical management,
chemotherapy with
vincristine, and
intralesional injection of absolute
ethanol can achieve good results in most of the patients with KMP.