Alveolar rhabdomyosarcoma with massive disseminated intravascular coagulopathy treated with systemic chemotherapy.
10.3345/kjp.2015.58.12.505
- Author:
Byung Gyu YOON
1
;
Hee Jo BAEK
;
Burm Seok OH
;
Dong Kyun HAN
;
Yoo Duk CHOI
;
Hoon KOOK
Author Information
1. Department of Pediatrics, Chonnam National University Hwasun Hospital, Chonnam National University Medical School, Hwasun, Korea. swan93@lycos.co.kr
- Publication Type:Case Report
- Keywords:
Rhabdomyosarcoma;
Disseminated intravascular coagulation;
Chemotherapy
- MeSH:
Adolescent;
Blood Platelets;
Blood Transfusion;
Child;
Cyclophosphamide;
Dacarbazine;
Disease Progression;
Disseminated Intravascular Coagulation;
Doxorubicin;
Drug Therapy*;
Drug Therapy, Combination;
Epistaxis;
Erythrocytes;
Hematuria;
Hemorrhage;
Humans;
Male;
Plasma;
Protease Inhibitors;
Rhabdomyosarcoma;
Rhabdomyosarcoma, Alveolar*;
Tissue Donors;
Vincristine
- From:Korean Journal of Pediatrics
2015;58(12):505-508
- CountryRepublic of Korea
- Language:English
-
Abstract:
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.
