미숙아에서 혈소판 감소증에 의해 발견된 장 혈관종 (Kasabach-Meritt 증후군) 1례
A Case of Intestinal Hemangioma Complicated with Thrombocytopenia (Kasabach-Merritt syndrome) in Premature Infant
2
조회
0
다운로드
0
인용
0
참고문헌
키워드
초록
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.
Abstract
Kasabach-Merritt syndrome is a rare thrombocytopenic consumptive coagulopathy associated with a giant hemangioma. We experienced a case of unexplained ascites with thrombocytopenia in a 32 week premature infant. An exploratory laparotomy was performed to determine the cause of the refractory ascites and thrombocytopenia. An intestinal hemangioma was found, but, surgical removal was not performed due to the extensive involvement. Hemangioma was confirmed by SPECT (single-photon emission computed tomography) and the thrombocytopenia was treated with steroid therapy. It is recommended that hemangioma of the visceral organs should be suspected when unexplained thrombocytopenia and disseminated intravascular coagulopathy persist.
참고문헌 (20)
- Herzog CE, "Benign vascular tumors. In: Nelson textbook of pediatrics. 18th ed", pp.2156~2157 (2007), WB Saunders Co
- Gascoyen GG, "Case of neavus involving the parotid gland, and causing death from suffocation: naevi of the viscera", Trans Pathol Soc Lond, 11, pp.267~ (1860)
- Ramanujam PS, "Hemangioma of the small intestine: case report and literature review", Am J Gastroenterol, 90, pp.2063~2064 (1995)
- Boyle L, "Solitary cavernous hemangioma of small intestine. Case report and literature review", Arch Pathol Lab Med, 117, pp.939~941 (1993)
- Kasabach HH, "Capillary hemangioma with extensive purpura: report of a case", Am J Dis Child, 59, pp.1063~1070 (1940)
- Margileth AM, "Developmental vascular abnormalities", Pediatr Clin North Am, 18, pp.773~800 (1971)
- Abbas AAH, "Haemangiomas: a review of the clinical presentations and treatment", Middle East Paediatrics, 8, pp.52~58 (2003)
- Weissman J, "Syndrome of hemangioma and thrombocytopenic purpura in infants", AMA Arch Intern Med, 92, pp.423~426 (1953)
- Scherz RG, "Giant hemangioendothelioma with associated thrombocytopenia", J Pediatr, 52, pp.212~216 (1958)
- Drolet BA, "Hemangiomas in children", N Engl J Med, 341, pp.173~181 (1999)
- Hatley RM, "Successful management of an infant with a giant hemangioma of the retroperitoneum and Kasabach-Merritt syndrome with alpha-interferon", J Pediatr Surg, 28, pp.1356~1359 (1993)
- Byard RW, "Diffuse infantile haemangiomatosis: clinicopathological features and management problems in five fatal cases", Eur J Pediatr, 150, pp.224~227 (1991)
- Gentry RW, "Vascular malformations and vascular tumors of the gastrointestinal tract", Surg Gynecol Obstet, 88, pp.281~323 (1949)
- Kwon YO, "A case of non-immune hydrops fetalis due to intraperitoneal hemangioma", J Korean Soc Neonatol, 12, pp.206~211 (2005)
- Landor M, "Images in clinical medicine. Hepatic hemangioma", N Engl J Med, 342, pp.791~ (2000)
- Enjolras O, "Management of alarming hemangiomas in infancy: a review of 25 cases", Pediatrics, 85, pp.491~498 (1990)
- Ozsoylu S, "Megadose methylprednisolone therapy for Kasabach-Merritt syndrome", J Pediatr, 129, pp.947~948 (1996)
- Hesselmann S, "Case report: Kasabach-Merritt syndrome: a review of the therapeutic options and a case report of successful treatment with radiotherapy and interferon alpha", Br J Radiol, 75, pp.180~184 (2002)
- Jones GJ, "Safety and tolerance of recombinant interferon alfa-2a (Roferon-A) in cancer patients", Cancer, 57, pp.1709~1715 (1986)
- Choi BS, "Hemangioma of the jejunum with mechanical ileus", J Korean Surg Soc, 28, pp.126~129 (1985)