Liver involvement in Hodgkin's lymphoma is common and is caused by hepatic infiltration, biliary obstruction by lymphoma, hepatitis, sepsis or complications of chemotherapeutic treatment. Jaundice caused by the vanishing bile duct syndrome related to Hodgkin's lymphoma is very rare. The mechanism is poorly understood but a paraneoplastic effect seems most likely as liver biopsy samples show cholestasis in the absence of lymphoma cells. Despite adequate treatment almost all reported patients died of liver failure or disease progression. Disease progression is explained partly by the difficulties encountered in the administration of potential hepatotoxic chemotherapy in severely cholestatic patients. We describe a 17-year-old man with vanishing bile duct syndrome and Hodgkin's lymphoma who was treated successfully with chemotherapy. The markedly elevated serum bilirubin levels completely normalized. Our case demonstrates that although dosing of chemotherapy in this situation can be very difficult, a good clinical outcome is possible, which makes the attempt at curative treatment worthwhile.
A 65-year-old female with primary biliary cirrhosis (PBC) presented with four hepatic lesions, visualized on a routinely performed ultrasound. She had no complaints. Clinical examination was normal. Laboratory investigation showed elevated liver tests, with a bilirubin level of 21 IU/l (normal <16 IU/l) and gamma-glutamyltransferase of 222 IU/l (normal < 37U/l). Alfa-fetoprotein was 3μg/l (normal < 9μg/l). Magnetic resonance imaging of the abdomen showed cirrhosis of the liver with lesions in segments II, III, IV and VIII. The lesions demonstrated high signal intensity on T2-weighted ( Fig. 1A) and diffusion imaging (Fig. 1B), including enhancement in the arterial phase after intravenous gadolinium administration, which raised suspicion of multifocal hepatocellular carcinoma. An extended left hepatectomy and radio frequency ablation were scheduled. During the laparotomy multiple lesions in the right liver and the ligamentum hepatoduodenale were found. Histology showed liver tissue with cirrhotic nodules and lymphatic lesions with populations of BCL2 positive B-cells, compatible with a marginal zone B-cell lymphoma (Fig. 1C). Staging showed an identical lymphoma in the bone marrow, compatible with stage IV non-Hodgkin's lymphoma (NHL). The patient was not responsive to immuno-chemotherapy and died after several weeks.Hepatic involvement of NHL can cause an elevation of liverassociated enzymes due to diffuse infiltration, but typically does not result in hepatic dysfunction and very rarely manifests as circumscribed lesions. Therefore, NHL is often misdiagnosed as primary liver cancer, metastases, focal nodular hyperplasia IMAGE OF THE ISSUEor abscesses. The risk for lymphoma in patients with PBC is negligible. Panjala et al. demonstrated that in 2,193 PBC patients only 13 cases were diagnosed with lymphoma [1].The short survival of NHL with major liver involvement makes early diagnosis essential [2]. Due to better imaging, molecular and serological tests and the concern for the development of port-side metastases [3], less biopsies are performed. If the clinical picture is suspicious for NHL, a liver biopsy should be attempted.
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