Presentation of Primary Hepatic Lymphoma (PHL) as Episodic Jaundice

Abstract

Case Presentation: A 27 yr-old male with 1-year history of episodic jaundice presented with 10-kilograms weight loss and jaundice over one month. His past medical history was relevant for an episode of alcoholic hepatitis when his symptoms of jaundice initially started. On presentation, he had generalized icterus and a large firm, mildly tender nodular liver. Blood tests showed mild coagulopathy along with moderately elevated hepatic enzymes. CT of the abdomen showed a 10 cm liver mass along with 2 smaller lesions in the left hepatic lobe. There was significant intrahepatic biliary ductal dilatation along with compression and thrombosis of portal vein. The excisional biopsy led to the diagnosis of diffuse B cell lymphoma based on immunoperoxidase staining. PET scan showed enhancing mass in the medial and lateral segments of left lobe of the liver. There was an increased activation in the area of retroperitoneal lymph nodes and the vertebral bodies T12 and L1 likely representing local metastases without any evidence of distant metastases or another primary. In view of the age and functional status, it was decided to give him curative doses of chemotherapy. After one cycle of chemotherapy his weight and functional status improved. Discussion: PHL is reported to occur with increased frequency in patients with chronic hepatitis C infection. Imaging studies reveal solitary, or less often, multiple masses in the liver. The predominant histology is B-cell lymphoma, most commonly diffuse large cell type. Most patients are treated with chemotherapy, with some physicians employing a multimodality approach incorporating surgery and radiotherapy with chemotherapy. The prognosis is variable, with good response to early aggressive combination chemotherapy regimens. The overall survival after surgical management can reach up to 10 yrs. PHL is a rare entity. It represents 0.4% of extranodal Non Hodgkin's lymphoma (NHL) and 0.016% of all NHL. The liver is involved in 15–27% in NHL. Hence it is important to rule out the involvement of other organ sites or disseminated disease to liver before considering the possibility of PHL. As with the majority of PHL, the lymphoma in the above case originated from B lymphocyte lineage. Also, the role of episode of alcoholic hepatitis in initiation of primary hepatic lymphoma is uncertain and it needs to be investigated.