Successful Management of Visceral Kaposiform Hemangioendothelioma with Kasabach‐Merritt Phenomenon Using Corticosteroids and Vincristine
2
조회
0
다운로드
1
인용
0
참고문헌
키워드
초록
Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular neoplasm that often develops a coagulopathy known as Kasabach-Merritt phenomenon (KMP). Visceral involvement denotes a poor prognosis. We report a case of visceral KHE with KMP successfully treated with corticosteroids and vincristine. The infant had been born vaginally at 34 weeks 6 days’ gestation, weighing 2,360 g. He was admitted for the management of respiratory failure. Blood tests showed anemia and thrombocytopenia 1 hour after delivery. Additional blood tests revealed a prothrombin time of 12.1 seconds, activated partial thromboplastin time of 60.6 seconds, fibrinogen levels of 72.4 mg/dL, and D-dimer levels >3,200 ng/mL. Despite supportive measures and daily transfusions, the clinical condition and coagulopathy gradually worsened. Renal ultrasonography performed to find the origin of the coagulopathy revealed an echogenic mass measuring >3 cm in the abdominal cavity. A magnetic resonance imaging scan showed an ill-marginated, infiltrative mass like lesion in the right anteromedial and posteromedial perirenal space that was hypointense with mild enhancement on T1- and T2-weighted images. Large vascular tumors adherent to other visceral organs were noted during exploratory laparotomy but could not be resected. Treatment with methylprednisolone was ineffective. Vincristine was administered weekly from the 17th hospital day, and the coagulation profile showed gradual improvement after its initiation. Intravenous methylprednisolone was switched to oral prednisolone on the 57th hospital day. He was discharged on the 73rd hospital day and continued vincristine treatment every 2 weeks and oral prednisolone administration as an outpatient treatment for 8 weeks. He remained symptom-free at the 39-month follow-up.
Abstract
Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular neoplasm that often develops a coagulopathy known as Kasabach-Merritt phenomenon (KMP). Visceral involvement denotes a poor prognosis. We report a case of visceral KHE with KMP successfully treated with corticosteroids and vincristine. The infant had been born vaginally at 34 weeks 6 days’ gestation, weighing 2,360 g. He was admitted for the management of respiratory failure. Blood tests showed anemia and thrombocytopenia 1 hour after delivery. Additional blood tests revealed a prothrombin time of 12.1 seconds, activated partial thromboplastin time of 60.6 seconds, fibrinogen levels of 72.4 mg/dL, and D-dimer levels >3,200 ng/mL. Despite supportive measures and daily transfusions, the clinical condition and coagulopathy gradually worsened. Renal ultrasonography performed to find the origin of the coagulopathy revealed an echogenic mass measuring >3 cm in the abdominal cavity. A magnetic resonance imaging scan showed an ill-marginated, infiltrative mass like lesion in the right anteromedial and posteromedial perirenal space that was hypointense with mild enhancement on T1- and T2-weighted images. Large vascular tumors adherent to other visceral organs were noted during exploratory laparotomy but could not be resected. Treatment with methylprednisolone was ineffective. Vincristine was administered weekly from the 17th hospital day, and the coagulation profile showed gradual improvement after its initiation. Intravenous methylprednisolone was switched to oral prednisolone on the 57th hospital day. He was discharged on the 73rd hospital day and continued vincristine treatment every 2 weeks and oral prednisolone administration as an outpatient treatment for 8 weeks. He remained symptom-free at the 39-month follow-up.
참고문헌 (20)
- Fletcher CDM, "WHO classification of tumours of soft tissue and bone", pp.110~111 (2013), International Agency for Research on Cancer
- Fernandez Y, "Kaposiform hemangioendothelioma", Eur J Intern Med, 20, pp.106~113 (2009)
- Nakaya T, "Multifocal kaposiform hemangioendothelioma in multiple visceral organs : an autopsy of 9-day-old female baby", Hum Pathol, 45, pp.1773~1777 (2014)
- Kasabach HH, "Capillary hemangioma with extensive purpura : report of a case", Am J Dis Child, 59, pp.1063~1070 (1940)
- Gilon E, "Multiple hemangiomata associated with thrombocytopenia: remarks on the pathogenesis of the thrombocytopenia in this syndrome", Blood, 14, pp.74~79 (1959)
- Brizel HE, "Giant hemangioma with thrombocytopenia. Radioisotopic demonstration of platelet sequestration", Blood, 26, pp.751~764 (1965)
- Seo SK, "Kasabach-Merritt syndrome:identification of platelet trapping in a tufted angioma by immunohistochemistry technique using monoclonal antibody to CD61", Pediatr Dermatol, 16, pp.392~394 (1999)
- Hall GW, "Kasabach-Merritt syndrome : pathogenesis and management", Br J Haematol, 112, pp.851~862 (2001)
- Pascal S, "Successful surgical management of congenital Kasabach-Merritt syndrome", Pediatr Int, 59, pp.89~92 (2017)
- Drolet BA, "Consensus-derived practice standards plan for complicated Kaposiform hemangioendothelioma", J Pediatr, 163, pp.285~291 (2013)
- Kim MG, "Kaposiform hemangioendothelioma of the breast in an adult female", Clin Breast Cancer, 11, pp.135~137 (2011)
- Drucker AM, "Vincristine and corticosteroids as first-line treatment of Kasabach-Merritt syndrome in kaposiform hemangioendothelioma", J Cutan Med Surg, 13, pp.155~159 (2009)
- Hauer J, "Effective treatment of kaposiform hemangioendotheliomas associated with Kasabach-Merritt phenomenon using four-drug regimen", Pediatr Blood Cancer, 49, pp.852~854 (2007)
- Barabash-Neila R, "Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon : successful treatment with vincristine and ticlopidine", Indian J Pediatr, 79, pp.1386~1387 (2012)
- Greinwald JH Jr, "An update on the treatment of hemangiomas in children with interferon alfa-2a", Arch Otolaryngol Head Neck Surg, 125, pp.21~27 (1999)
- Lopez V, "Successful management of Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon using vincristine and ticlopidine", Pediatr Dermatol, 26, pp.365~366 (2009)
- Mizutani K, "Successful combination therapy of propranolol and prednisolone for a case with congenital Kasabach-Merritt syndrome", J Dermatol, 44, pp.1389~1391 (2017)
- Cashell J, "Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon in an infant : Successful treatment with prednisolone, vincristine, and addition of sirolimus", Pediatr Blood Cancer, 65, pp.e27305~ (2018)
- Garcia-Monaco R, "Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon : successful treatment with embolization and vincristine in two newborns", J Vasc Interv Radiol, 23, pp.417~422 (2012)
- 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)