Diffuse Large B-cell Lymphoma - A Rare Cause of Acute Liver Failure

Abstract

Acute liver failure is a rare but life-threatening illness with an incidence of 2-8 per million populations. Most common causes of ALF include drug-induced liver injury, viral hepatitis, autoimmune disease, metabolic diseases and very rare malignancy. Malignant infiltration of the liver is a rare presentation of acute liver failure associated with poor prognosis. We report a case of a patient with acute liver failure caused by malignant infiltration by diffuse large B-cell lymphoma. A 28-year-old female with no significant past medical history or social history presented with abdominal distention and jaundice for four days associated with increased fatigue. She denied weight loss, fever, or night sweats. On physical examination, the patient had scleral icterus, distended non-tender abdomen with massive hepatosplenomegaly and minimal hepatic encephalopathy. Initial laboratory work was notable for; ALT 53 IU/L, AST 394 IU/L, Alkaline Phosphatase 433IU/L, Direct Bilirubin 7.4mg/dl, total Bilirubin 11.4mg/dl, PT 17.6secs, INR 1.6, PTT 28.2 secs. Abdominal Ultrasound showed an enlarged diffusely, heterogeneous liver concerning for metastasis or primary hepatic malignancy. An abdominal Computed Tomography scan revealed massive hepatosplenomegaly, heterogeneous hepatic involvement, with lymphomatous involvement of abdominal, pelvic and inguinal lymph nodes. Workup for his acute liver failure including viral, autoimmune and metabolic disease was unrevealing. Patient subsequently underwent an inguinal core lymph node biopsy that showed high-grade lymphoma (CD10-, MUM1-), double expression (IHC +, C MYC, BCL6), high replication rate- Ki-67 80-90%, IPI 3, 85% CD5+, CD10+ and CD 20+, as well as weakly positive for CD19 (Figure 1). The patient was started on chemotherapy, which consisted of hyper CVAD (Vincristine, Rituximab, Cyclophosphamide and Dexamethasone. Initial treatment was well tolerated and the patient was discharged for out-patient continuation of chemotherapy. Acute liver failure is characterized by the development of severe liver injury with impaired synthetic capacity and encephalopathy in patients with a previously normal liver. Acute liver failure due to malignant infiltration is very rare, this case highlights a high index of suspicion, particularly if no obvious etiology identified. Malignancy causing acute hepatic failure must be recognized to avoid inappropriate referral for transplantation and delay in initiating treatment.2342_A Figure 1. Inguinal core lymph node biopsy showing high-grade B-cell lymphoma IHC positive for C-MYC and BCL6.2342_B Figure 2. Axial CT abdomen with massive hepatomegaly (red arrow) and splenomegaly (blue arrow)2342_C Figure 3. Coronal CT abdomen with massive hepatomegaly (red arrow) and splenomegaly (blue arrow)