Primary Liver Sinusoidal Non-Hodgkin's Lymphoma Presenting as Acute Liver Failure

Abstract

We describe a case of a middle-aged woman, who presented to us with fever, anorexia, abdominal distension from a massive hepatomegaly, low hemoglobin, and acute liver failure. A liver biopsy revealed B cell non-Hodgkin's lymphoma predominantly in the sinusoids with CD10, CD20, and Bcl-2 positive on immunohistochemistry. She initially responded well to chemotherapy but succumbed 6 months later to the recurrence of disease. Sinusoidal non-Hodgkin's lymphoma of the liver should be considered in the differential diagnosis of a patient with large hepatomegaly presenting with acute liver failure. ( J CLIN EXP HEPATOL 2015;5:341-343) M alignancies of the liver are known to rarely cause acute liver failure. 1,2 We describe a rare primary malignancy of the liver presenting with acute liver failure.A 53-year-old housewife presented with history of fever, anorexia, and abdominal distension of 2 weeks duration. She was well prior to the onset of these symptoms. She gave history of being treated for carcinoma breast (infiltrating ductal carcinoma of the breast) five years back with surgery (modified radical mastectomy) and chemotherapy (Paclitaxel-based regimen with Tamoxifen). She denied any chest pain, abdominal pain, vomiting, and diarrhea, blood in stools, night sweats and weight loss. Clinically she had pallor and massive firm hepatomegaly, palpable up to the right iliac fossa. There was no evidence of lymphadenopathy or stigmata of chronic liver disease.Her complete blood count showed hemoglobin of 5.6 g %, WBC count of 4970/cmm and platelet count of 1,70,000/mm. Her liver profile showed bilirubin of 2.5 mg/dl, ALT and AST of 165 and 186 U/L respectively, alkaline phosphatase of 217 U/L (normal range 30-150), serum albumin of 3.8 g/dl, INR of 1.7 with normal creatinine and serum electrolytes. Her HBsAg, anti-HCV, anti-EBV IgM and HIV serology were negative. Serum AFP was normal. Contrast enhanced computed tomography (CT) showed massive hepatomegaly with no focal lesions, splenomegaly with minimal bilateral pleural effusion and minimal fluid in the pelvis.On the second day of admission, she developed altered sensorium and drowsiness with high arterial ammonia of 140 mmol/L (normal range 9-33).A percutaneous liver biopsy was performed under cover of fresh frozen plasma. Her liver biopsy revealed hepatic sinusoidal B cell non-Hodgkin's lymphoma, which on immunochemistry showed CD10 and CD20 positivity (implying lymphocyte germinal center origin) and Bcl 2 positivity (implying B cell origin) (Figures 1-3). The cyto keratin (CK) marker on immunohistochemistry was negative thereby ruling out metastases from the previous breast cancer. Bone marrow aspiration did not show any involvement by the lymphoma.She received cyclophosphamide, adriamycin, vincristine, and prednisolone (CHOP) regimen with supportive care including anti-coma measures, hydration and blood products. Within 48 h of initiation of treatment, she showed improvement in sensorium and regression of liver span by 5 cm. She had a drop in her leukocyte count after...

Primary hepatic peripheral T-cell lymphoma associated with Epstein-Barr viral infection

“…Abnormal laboratory findings include elevated liver function aminotransferases, bilirubin, γ-glutamyl transferase, ALP and LDH. Approximately 70% of cases present with abnormal liver function enzymes, 30 to 80% with elevated LDH, 90% with elevated β2-microglobulin, and 80% with elevated ALP[ 17 - 20 ]. Mitarnun et al[ 20 ] reported that out of 100 patients with EBV associated H-PTCL, ALP was found elevated in 80%, while LDH was elevated in 65% of cases.…”