Primary hepatic non-Hodgkin's lymphomas: case report and review of the literature

Abstract

A 31-yr-old man presented with a 1-wk history of fever, chills, weakness, headaches, and a significant 20-lb weight loss over the preceding 2 months. His past medical history was relevant for liver amebiasis during childhood. Two days before admission, the patient noticed jaundice. He denied abdominal pain or other GI symptoms, and there was no history of alcohol intake, medications, or illicit drugs. His physical examination revealed generalized jaundice, hepatosplenomegaly, and bilateral leg edema. Neurologically, the patient was agitated, with periods of disorientation, and he had bilateral flapping. His blood tests revealed pancytopenia, renal failure, liver failure, and coagulopathy. Because the patient had a fever, hepatosplenomegaly, and pancytopenia, a further workup also included a bone marrow and liver biopsy. No conclusive diagnosis could be made from the above tests, and the patient died 5 days after admission. Postmortem evaluation, including flow cytometry and gene rearrangement in the tissue obtained from the liver, revealed large B cell lymphoma. This case illustrates an unusual presentation of hepatic non-Hodgkin's lymphoma. Current information regarding this entity is scant, mainly owing to its rarity. We present a review of the literature, including the incidence, presentation, treatment, and prognosis of primary hepatic lymphoma.

Non-Hodgkin Lymphoma of the Liver: A US Population-based Analysis

“…NHL is more likely than Hodgkin lymphoma to have hepatic involvement 3. The prevalence of liver involvement by NHL is reported to be 15% to 30%, and more than 50% in necropsy studies 3–8…”

“…PHL represents 0.4% of extranodal NHLs and 0.016% of all NHLs 7,8. Based on liver infiltration, PHL can be subdivided into uninodular (39–42%), multinodular (50–55%), and diffuse (6–8%) types 6,9.…”

“…Chronic antigen stimulation by viral infection (HIV, hepatitis C virus (HCV), hepatitis B virus (HBV) and Epstein-Barr virus (EBV), liver cirrhosis, immunosuppressive therapy and autoimmune disease are implied to have an etiological relevance to the development of PHL 8. HCV is found in 10% to 60% of patients with PHL,6,9,13,14 with geographic variations, and these patients have aggressive histological subtypes 9,15.…”

“…PHL most commonly affects men during their fifth decade of life, and has a male-to-female ratio of 2–3:1 3,8,17. Liver biopsy is the most valuable tool for diagnosis 10,14.…”

“…Incidental identification has been documented in 10% of PHL cases 18. Acute liver failure reportedly accounts for 4.5% of PHL presentations,8 and about 3% of reported acute liver failure was related to hepatic lymphoma,19 notably of diffuse pattern 20. Most cases of acute liver failure from lymphoma are diagnosed on autopsy, with an average survival of 11 days from diagnosis 21.…”