Favorable response to treatment of a child with T-cell-rich large B-cell lymphoma presenting with liver failure

The authors describe the successful management of a child with T-cell-rich large B-cell lymphoma (TCRBCL) involving the lymph nodes and liver, causing severe hepatic dysfunction. After immunohistochemical confirmation of the diagnosis, the patient was treated initially with low-dose, non-hepatotoxic chemotherapy and irradiation to the porta hepatis. Chemotherapy was gradually escalated to intensified B cell-lymphoma treatment regimens (CHOP, CYVE, COPAdM) as liver function improved. Despite prompt and notable response in the lymph nodes, the intrahepatic nodular lesions persisted radiologically. Liver function tests normalized during the course of treatment. However, radiologic studies of the liver showed abnormal regeneration with a predominant left lobe overshadowing a tiny right lobe. The patient remains in remission more than a year after completion of treatment. Judicious use of chemotherapeutic agents with or without local radiotherapy may influence the outcome and survival in children with TCRBCL involving the liver and causing abnormal hepatic function.