Acute Hepatomegaly: A Rare Manifestation of Richter Transformation Following Elective Splenectomy for Splenic Marginal Zone Lymphoma

Abstract

Richter transformation represents an the development of an acute aggressive lymphoma manifesting in a sudden onset of B symptoms, with enlarging lymph nodes felt on physical exams or seen on imaging in the setting of underlying chronic lymphoma. These changes can be seen biochemically with a substantially elevated lactate dehydrogenase level as well as abnormalities in cell lineages. The triggers behind transformation are far from understood. Identified risk factors that have been evaluated include chemotherapy regimen, transformative viral infections, and individual genetic mutations. We present the case of transformation from an indolent marginal zone lymphoma to high grade lymphoma with hepatic infiltration following splenectomy without any associated risk factors. Our patient is a 46 year old female with marginal zone lymphoma who presented to the ED with severe abdominal pain which started 1 week after elective splenectomy. Originally discharged from ED the night prior, she returned when her pain worsened. Imaging from initial ED encounter showed acute hepatomegaly with diffuse lymphadenopathy. Labs demonstrated marked transaminasemia, worsening coagulopathy, and severe lactic acidosis. Patient was transferred to the ICU where she underwent liver and bone marrow biopsy which were conclusive for DLBCL. PICC line was placed and patient was started on emergent R-CHOP therapy the same evening. Unfortunately, she developed tumor lysis syndrome complicated by abdominal compartment syndrome and ultimately transferred to comfort care and terminally extubated. Our case highlights the lack of knowledge regarding RT. While the consensus opinion in literature identifies a number of internal and external risk factors, our patient did not have any of the risk factors; she never had chemotherapy, her pre-operative titers were negative for acute CMV and EBV infection. More curious is the liver infiltration following splenectomy. A review of the available literature does not mention liver infiltration in the setting of acute Richter transformation. The complications of management due to acute liver failure including refractory acidosis and impaired gluconeogenesis in the setting of tumor lysis syndrome following chemotherapy dramatically decreased odds of survival. We propose liberal follow up for any patient diagnosed with an indolent lymphoma with accompanying B symptoms given the unpredictability of Richter Transformation.2372_A Figure 1. CT scan showing hepatomegaly that developed two weeks following splenectomy. Pre-op CT demonstrated normal sized liver.2372_B Figure 2. H&E stain and Ki67 index of spleen removed prior to admission noted for nodular architecture of neoplastic B-cells. Ki-67 index of ˜50% without sheets of large shells to diagnose transformation to large cell lymphoma.2372_C Figure 3. Liver biopsy with extensive involvement of high grade NHL in hepatic parenchyma. Markedly increased proliferative infdex by Ki-67 in transformation to high grade lymphoma.