Clinical and Experimental Pediatrics

대한소아청소년과학회 (The Korean Pediatric Society)
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Alveolar rhabdomyosarcoma with massive disseminated intravascular coagulopathy treated with systemic chemotherapy

  • Yoon, Byung Gyu (Department of Pediatrics, Chonnam National University Hwasun Hospital, Chonnam National University Medical School) ;
  • Baek, Hee Jo (Department of Pediatrics, Chonnam National University Hwasun Hospital, Chonnam National University Medical School) ;
  • Oh, Burm Seok (Department of Pediatrics, Chonnam National University Hwasun Hospital, Chonnam National University Medical School) ;
  • Han, Dong Kyun (Department of Pediatrics, Chonnam National University Hwasun Hospital, Chonnam National University Medical School) ;
  • Choi, Yoo Duk (Department of Pathology, Chonnam National University Hwasun Hospital, Chonnam National University Medical School) ;
  • Kook, Hoon (Department of Pediatrics, Chonnam National University Hwasun Hospital, Chonnam National University Medical School)
  • 투고 : 2013.10.25
  • 심사 : 2014.05.16
  • 발행 : 2015.12.10
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초록

It is uncommon for pediatric patients with rhabdomyosarcoma to present with clinical and/or laboratory features of disseminated intravascular coagulation (DIC). We report a case of metastatic alveolar rhabdomyosarcoma with severe bleeding because of DIC in a 13-year-old boy. He experienced persistent oozing at the site of a previous operation, gross hematuria, and massive epistaxis. Two weeks after initiating combination chemotherapy consisting of vincristine, doxorubicin, and cyclophosphamide, the patients' laboratory indications of DIC began to resolve. During this period, the patient received massive blood transfusion of a total of 311 units (26 units of red blood cells, 26 units of fresh frozen plasma, 74 units of platelet concentrates, 17 units of single donor platelets, and 168 units of cryoprecipitate), antithrombin-III and a synthetic protease inhibitor. Despite chemotherapy and radiation therapy, he died 1 year later because of disease progression. In children with metastatic rhabdomyosarcoma and massive DIC, prompt chemotherapy and aggressive supportive care is important to decrease malignancy-triggered procoagulant activities.

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참고문헌

  1. Bick RL, Arun B, Frenkel EP. Disseminated intravascular coagulation. clinical and pathophysiological mechanisms and manifestations. Haemostasis 1999;29:111-34.
  2. Levi M. Disseminated intravascular coagulation in cancer patients. Best Pract Res Clin Haematol 2009;22:129-36. https://doi.org/10.1016/j.beha.2008.12.005
  3. Sallah S, Wan JY, Nguyen NP, Hanrahan LR, Sigounas G. Disseminated intravascular coagulation in solid tumors: clinical and pathologic study. Thromb Haemost 2001;86:828-33. https://doi.org/10.1055/s-0037-1616139
  4. Barbui T, Falanga A. Disseminated intravascular coagulation in acute leukemia. Semin Thromb Hemost 2001;27:593-604. https://doi.org/10.1055/s-2001-18865
  5. Bien E, Maciejka-Kapuscinska L, Niedzwiecki M, Stefanowicz J, Szolkiewicz A, Krawczyk M, et al. Childhood rhabdomyosarcoma metastatic to bone marrow presenting with disseminated intravascular coagulation and acute tumour lysis syndrome: review of the literature apropos of two cases. Clin Exp Metastasis 2010;27:399-407. https://doi.org/10.1007/s10585-010-9335-y
  6. Weltermann A, Mitterbauer GJ, Mitterbauer M, Hattey E, Speiser W, Gisslinger H, et al. Disseminated intravascular coagulation (DIG) with massive hyperfibrinolysis in metastatic uterine cancer. Observations on the effects on the coagulopathy of various treatments (a case report). Wien Klin Wochenschr 1998;110:53-7.
  7. Levi M, Opal SM. Coagulation abnormalities in critically ill patients. Crit Care 2006;10:222. https://doi.org/10.1186/cc4975
  8. Pernerstorfer T, Hollenstein U, Hansen J, Knechtelsdorfer M, Stohlawetz P, Graninger W, et al. Heparin blunts endotoxin-induced coagulation activation. Circulation 1999;100:2485-90. https://doi.org/10.1161/01.CIR.100.25.2485
  9. De Cicco M. The prothrombotic state in cancer: pathogenic mechanisms. Crit Rev Oncol Hematol 2004;50:187-96. https://doi.org/10.1016/j.critrevonc.2003.10.003
  10. Teh RW, Tsoi DT. Acute disseminated intravascular coagulation in neuroendocrine carcinoma. Case Rep Oncol 2012;5:524-9. https://doi.org/10.1159/000338401

피인용 문헌

  1. Alveolar rhabdomyosarcoma with bone marrow metastasis causes disseminated intravascular coagulation vol.2, pp.4, 2018, https://doi.org/10.1002/ped4.12102