• Archives of Plastic Surgery
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• 제32권5호
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• pp.648-652
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• 2005

In 1940, Kasabach and Merritt first described the association of a large vascular tumor and thrombocytopenia and termed this Kasabach-Merritt(KM) syndrome. It is characterized by a rapidly enlarging vascular anomaly and consumptive coagulopathy with thrombocytopenia, prolonged prothrombin time and partial thromboplastin time, hypofibrinogenemia, and the presence of D-dimer and fibrin split product, with or without microangiopathic hemolytic anemia. This is a potentially life-threatening condition with mortality rates from 20 to 30% as a result of severe sepsis, coagulopathy, or invasion of vital organs. Treatment modalities are corticosteroids, interferon alfa-2a or 2b, chemotherapy(vincristine, cyclophosphamide, etc.), aspirin, dipyridamole, com- pression, radiation therapy, embolization of feeding vessels and surgical excision. A standard treatment regimen for KM syndrome has not been established and most reports on definitive management of these complex vascular lesions have been anecdotal, involving small numbers of patients. The authors have successfully treated a patient of KM syndrome with actively bleeding huge hemangioma by surgical excision. They present it with the review of articles.

• Neonatal Medicine
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• 제17권1호
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• pp.116-122
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• 2010

Kasabach-Merritt 증후군은 거대한 혈관종에 의해 혈소판이 소모되어 감소되는 드문 질환이다. 저자들은 재태주령 32주 미숙아에서 설명되지 않은 복수와 혈소판 감소증의 원인을 찾기 위해 시험적 개복술을 시행하여 장 혈관종을 발견하였으나 광범위하여 수술적 제거를 하지 못하고, SPECT로 확인 후 스테로이드 치료로 혈소판 감소증이 호전된 1례를 경험하였다. 따라서 다른 원인에 의해 설명되지 않는 혈소판 감소증과 파종성 혈관내 응고병증이 있으면 내부 장기의 혈관종을 의심해 볼 필요가 있다고 사료되었다.

• Nuclear Medicine and Molecular Imaging
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• 제43권1호
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• pp.83-86
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• 2009

Kasabach-Merritt syndrome (KMS) consists of thrombocytopenia, microangiopathic hemolytic anemia, and localized consumption coagulopathy that develops within vascular hemangioma. This syndrome may also be associated with occult hemangiomas located at various sites. Tc-99m RBC scintigraphy and SPECT have proven to be reliable for confirming or excluding hemangioma. Total body blood pool imaging study during the scintigraphy also provides a means of screening for occult lesions. The authors report the case of a 29-year-old man who presented with a giant hepatic hemangioma complicated by KMS, and underwent Tc-99m RBC scintigraphy and SPECT including a total body blood pool imaging study.

• Clinical and Experimental Pediatrics
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• 제48권9호
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• pp.1004-1008
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• 2005

혈관종은 영아에서 가장 흔한 양성 종양으로 이 중 15-30%는 다발성 혈관종을 보인다. 미만성 신생아 혈관종증은 피부와 다수의 내부 장기를 침범하는 질환으로 치료하지 않는 경우 치명적일 수 있기 때문에 신속하고 적극적인 치료가 요구된다. 치료는 부신피질 호르몬이 1차 선택 약제로 사용되며 이에 반응하지 않으면 인터페론, 절재술, 전색술, 방사선 치료 등을 이용할 수 있다. Interferon alfa-2a는 매우 효과적이나 강직성 양마비(spastic diplegia)와 같은 심각한 합병증이 보고되고 있다. 저자들은 출생 직후 호흡곤란과 전신의 피부 혈관종을 주소로 입원하여 Kasabach-Merritt 증후군을 동반한 미만성 신생아 혈관종증을 진단 받았던 1례에서 스테로이드 치료에 대해 혈소판 감소증 및 소모성 응고장애의 호전은 있었으나 혈관종의 수 및 크기의 호전이 없어 vincristine을 투여하여 치료에 성공하였기에 문헌 고찰과 함께 보고하는 바이다.

• Advances in pediatric surgery
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• 제14권2호
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• pp.134-143
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• 2008

Infantile hepatic hemangioendothelioma (IHHE) is the most common benign vascular hepatic tumor in children. We analyzed the 17-year experience of IHHE. The medical records of 16 patients (M:F=8:8) treated at the Department of Pediatric Surgery and the Department of Pediatrics Seoul National University Children's Hospital between January 1991 and January 2008 were reviewed retrospectively. Mean age at presentation was 87 days (1 day - 551 days). Seventy five percent of patients were diagnosed with imaging study and 25 % with biopsy. Major symptoms were hepatomegaly (N=5), palpable abdominal mass (N=4) and congestive heart failure (N=3). Six patients had no symptoms. Kasabach-Merritt syndrome was combined in one patient. Nine patients (56.3 %) underwent operation and 2 patients (12.5 %) underwent only medical treatment. Clinical observation was tried on 5 patients (31.3 %) without any treatment. Operation was performed on the patient with clinical symptoms or on patients where the differentiation between begin and malignant could not be determined. Patients who had clinical symptoms but tumor was unreresectabile were treated medically. Among the 5 patients who had been observed for their clinical course, 2 patients showed complete regression and the tumors of the remaining 3 patients were regressing. Clinical symptoms, the age at presentation, the size of tumor and ${\alpha}$-FP, all had no significant statistical relationship with the time required for complete tumor regression. There was no relationship between the size change of the tumor and the change of ${\alpha}$-FP level. Only the size of tumor was related with clinical symptoms. One patient died of post-operatvie bleeding. Treatment plan was determined by the extent of the tumor and the presence of clinical symptoms. Observation was enough for the patients without clinical symptoms and complete resection was curative for patients with clinical symptoms. Medical treatment is an alternative for the patient whose tumor is unresectable.