Cancer Res Treat.  2016 Oct;48(4):1443-1447. 10.4143/crt.2016.096.

Severe Hepatic Sinusoidal Obstruction Syndrome in a Child Receiving Vincristine, Actinomycin-D, and Cyclophosphamide for Rhabdomyosarcoma: Successful Treatment with Defibrotide

• Choi A
• Kang YK
• Lim S
• Kim DH
• Lim JS
• Lee JA

• KMID: 2356247
• DOI: http://doi.org/10.4143/crt.2016.096

Abstract

Hepatic sinusoidal obstruction syndrome (SOS) is a life-threatening syndrome that generally occurs as a complication after hematopoietic stem cell transplantation or, less commonly, after conventional chemotherapy. Regarding SOS in rhabdomyosarcoma patients who received conventional chemotherapy, the doses of chemotherapeutic agents are associated with the development of SOS. Several cases of SOS in rhabdomyosarcoma patients after receiving chemotherapy with escalated doses of cyclophosphamide have been reported. Here, we report on a 9-year-old female with rhabdomyosarcoma who developed severe SOS after receiving chemotherapy consisting of vincristine, actinomycin-D, and a moderate dose of cyclophosphamide. She was treated successfully with defibrotide without sequelae to the liver.

Keyword

Hepatic veno-occlusive disease; Rhabdomyosarcoma; Defibrotide

MeSH Terms

Child*
Cyclophosphamide*
Drug Therapy
Female
Hematopoietic Stem Cell Transplantation
Hepatic Veno-Occlusive Disease*
Humans
Liver
Rhabdomyosarcoma*
Vincristine*
Cyclophosphamide
Vincristine

Figure

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  Fig. 1. Abdominal Doppler ultrasonography performed on hospital day (HD) 3 (A) and HD 22 (B). Blood flow of the right portal vein was reversed (A) upon sinusoidal obstruction syndrome diagnosis. Direction of blood flow was normalized (B) after treatment with defibrotide.

• 

  Fig. 2. Treatment and progress. AC, abdominal circumference; WBC, white blood cell; AST, aspartate transaminase; ALT, alanine transaminase; PLT, platelet; T.bil, total bilirubin; HD, hospital day; UDCA, ursodeoxycholic acid.

Reference

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