Abstract
We have recently observed 3 children with AML treated with daunorubicin (DNR), ara-c and thioguanine (TG) who during remission developed marked hepatomegaly (H) in the absence of jaundice or significant transaminasemia. Liver scans revealed diffuse non-homogeneous reduction in uptake suggesting leukemic infiltration. Liver biopsies showed no significant infiltration, but instead thickening of the walls of the central veins, and intense centrllobular congestion and hemorrhage, a pattern resembling VOD caused by plant alkaloid poisoning. One child had a large R. pleural effusion requiring chest drainage for 1 month. All 3 children have been since maintained only on TG. H and abnormal liver scans have improved but remain abnormal 8-10 months later. One child in relapse was re-treated with all 3 drugs and developed an exacerbation of the liver disease and pleural effusion. The above reactions all occurred within a 2 month period in 1976 and had not been seen previously in other children receiving the same drug regimen. Etiologic considerations include an altered lot of DNR, enhanced toxicity of DNR by TG-induced hepatic dysfunction, or other complex interactions. Recognition of this type of liver disease is important in the diagnosis of acute hepatomegaly in such children. Further elucidation of its pathogenesis may enable safer usage of these potent drug combinations.
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Naiman, J., Wimmer, R., Huff, D. et al. VENO-OCCLUSIVE DISEASE (VOD): A NEWLY RECOGNIZED FORM OF DRUG-INDUCED LIVER DISEASE IN ACUTE MYELOCYTIC LEUKEMIA (AML). Pediatr Res 11, 478 (1977). https://doi.org/10.1203/00006450-197704000-00647
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DOI: https://doi.org/10.1203/00006450-197704000-00647