Korean J Pediatr.  2015 Dec;58(12):505-508. 10.3345/kjp.2015.58.12.505.

Alveolar rhabdomyosarcoma with massive disseminated intravascular coagulopathy treated with systemic chemotherapy

• Yoon BG
• Baek HJ
• Oh BS
• Han DK
• Choi YD
• Kook H

• KMID: 2135254
• DOI: http://doi.org/10.3345/kjp.2015.58.12.505

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.

Keyword

Rhabdomyosarcoma; Disseminated intravascular coagulation; Chemotherapy

MeSH Terms

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
Cyclophosphamide
Dacarbazine
Doxorubicin
Protease Inhibitors
Vincristine