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Hepatic sinusoidal obstruction syndrome in a child: case report A 9-year-old boy developed hepatic sinusoidal obstruction syndrome during treatment with prednisolone, vincristine, cyclophosphamide, daunorubicin, asparaginase, methotrexate, cytarabine and hydrocortisone [not all routes stated]. The boy was hospitalised and diagnosed with precursor B-cell lymphoblastic lymphoma of his right cerebral hemisphere. He received an induction therapy regimen based on prednisolone 60 mg/m2 daily for 5 weeks; vincristine 1.5 mg/m2 on days 8, 15, 22 and 29; cyclophosphamide 1.0 g/m2 on day 8; daunorubicin 40 mg/m2 on days 8, 15 and 22; asparaginase 6000 U/m2 on days 9, 11, 13, 16, 18, 20, 23, 25 and 27; and triple intrathecal therapy on days 1, 15 and 29 with methotrexate 12.5mg, cytarabine 25mg and hydrocortisone 25mg. Peripheral neuropathy with pain developed on day 27 of induction therapy. Induction therapy was withdrawn, with the exception of prednisolone. On day 29, his total bilirubin level was elevated to 2.1 mg/dL, and he had abdominal effusion and pain in his right upper quadrant. Abdominal ultrasound demonstrated an enlarged liver, a large volume of ascites, and retrograde flow in his right hepatic vein. His weight had increased by a further 5% from his pretreatment baseline on day 31. Hepatic sinusoidal obstruction syndrome was diagnosed. The boy received ursodeoxycholic acid and low molecular weight heparin. However, his clinical status was aggravated by a total bilirubin of 7.0 mg/dL, a further 4kg increase in his weight, decreased antithrombin III and protein C activity levels, and transfusion-refractory thrombocytopenia. He received antithrombin III concentrate. He became stuporous on day 34, with jaundice and abdominal distension. On day 50, his total bilirubin level was 21 mg/dL despite supportive therapy. He developed bradycardia and loss of the light reflex on day 55; brain CT scanning revealed intracranial haemorrhage. He died of brain herniation on day 56. Author comment: "[T]his case suggests that the standarddose administration of chemotherapeutic agents based on the actual weight was associated with fatal HSOS [hepatic sinusoidal obstruction syndrome]". Yamamoto S, et al. Fatal hepatic sinusoidal obstruction syndrome in a child with primary CNS lymphoma during induction therapy. International Journal of Hematology 96: 284-286, No. 2, Aug 2012. Available from: URL: http:// 803080801 dx.doi.org/10.1007/s12185-012-1128-4 - Japan

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Reactions 8 Dec 2012 No. 1431