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Title   Kasabach-Merritt Syndrome을 동반한 비장의 원발성 혈관육종 1예 ( A Case of Primary Splenic Angiosarcoma Associated with Kasabach-Merritt Syndrome )
Publicationinfo   1997 Jan; 029(02): 352-358.
Key_word   Splenic angiosarcoma, Kasabach-Merritt syndrome, High-dose chemotherapy
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Abstract   Purpose: Primary malignant vascular neoplasms of the spleen are rare. It has been known that the prognosis was very poor and the splenectomy before rupture could increase survival. No effective chemotherapeutic protocol for angiosarcomas has yet to be established but patients with or without metastatic disease may be treated by chemotherapy. Material and Mehtods: We experienced a case of prirnary splenic angiosarcoma in a 42-year-old woman with multiple purpuric skin rashes associated with consumptive coagulopathy:the Kasabach-Merritt syndrome. The CT showed spleen is diffusely enlarged and inhomogenously enhanced with multiple metastasis in the liver. The splenectomy was done and angiosarcoma was diagnosed. We treated her with conventional combination chemotherapy and obtained partial response. For additional response, high-dose chemotherapy and stem cell reseue with autologous peripheral blood stem cell transplantation was done. Result: Afer splenectomy, platelet count return to normal. The follow up abdominal CT scan after treatment showed complete disappeared multiple metastatic lesions in the both lobe of liver and the patient has continued to do well four months following discharge. Conclusion: We herein report our experience of a splenic angiosarcoa whose multiple hepatic metastases responded well to the high-dose chemotherapy.
저 자   전진석(Jin Seok Jun),이규택(Kyu Taek Lee),변재호(Jae Ho Byun),한기주(Ki Joo Han),박성규(Sung Kyu Park),원종호(Jong Ho Won),백승호(Seung Ho Baek),홍대식(Dae Sik Hong),박희숙(Hee Sook Park),이혜경(Hye Kyung Lee),진소영(So Young Jin)