S2584 A Rare Case of Primary Hepatic Diffuse Large B-Cell Lymphoma

Abstract

INTRODUCTION: Diffuse large B cell lymphoma is a common highly malignant type of non-Hodgkin's lymphoma that develops in the lymph nodes. Primary hepatic diffuse large B cell lymphoma (PHDLBCL), which accounts for 0.016% of all non-Hodgkin’s lymphomas, is a rare clinical condition that is confined to the liver only without the involvement of any other organ. In this case, we describe a young female who presented with abdominal pain and was diagnosed with PHDLBCL after extensive workup. CASE DESCRIPTION/METHODS: A 31 year-old African American female with history of smoking and human immunodeficiency virus presented with three weeks of nausea, vomiting, and persistent right upper quadrant (RUQ) abdominal pain. She denied any other systemic symptoms, sick contacts, or recent travel. On exam, the RUQ of her abdomen was tender to palpation. Labs were remarkable for hemoglobin of 6.9 g/dL, transaminases of AST 51 U/L, ALT 13 U/L, and alkaline phosphatase of 247. LDH was 1405 U/L and haptoglobin 272 mg/dL. CMV, EBV, HSV1, and HSV2 IgG serologies were all positive. Abdominal ultrasound demonstrated hepatomegaly with numerous “target shaped lesions” with the largest measuring 7.7 cm in the right hepatic lobe. Magnetic resonance imaging of the abdomen with contrast showed multiple thick-walled liver lesions. Nuclear medicine positron emission tomography scan performed showed numerous intensely FDG avid hepatic lesions with some demonstrating central necrosis, osseous lesions in axial and appendicular skeleton, and mildly enlarged thoracoabdominal lymph nodes. Liver biopsy revealed sheets of high-grade malignant lymphocytes, consistent with high grade B cell lymphoma, germinal center subtype. These cells stained positive for CD 20, pax 5, CD 10, Ki-67 and BCL-6. P53 staining was also diffusely strong and positive. She was initiated on etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin with concomitant administration of antiretroviral therapy. Rituximab was held given her low CD4 count. DISCUSSION: PHDLBCL is an extremely rare malignancy found in clinical practice and particularly occur in patients with underlying immunosuppression. It is very important to consider this disease and other infiltrative processes in the differential diagnosis of RUQ abdominal pain and abnormal liver function tests with unclear etiology. In these cases, early liver biopsy should be pursued. The overall prognosis of primary hepatic lymphomas remains poor and early recognition of this disease may improve the prognosis.Figure 1.: MR Abdomen with IV contrast demonstrating multiple intra-hepatic thick-walled liver lesions.Figure 2.: NM PET CT revealing intensely FDG avid numerous hepatic lesions with some demonstrating central necrosis.Figure 3.: Liver biopsy demonstrating the normal liver parenchyma being completely replaced by sheets of malignant lymphocytes.