Massive Splenomegaly as Initial Presentation of Diffuse Large B-Cell Lymphoma

Abstract

Diffuse large B-cell lymphoma (DLBCL) is the most common subtype of non-Hodgkin's lymphoma in western countries and is characterized by aggressive clinical behavior. Primary splenic DLBCL is an extremely rare clinical condition. We present a case of successfully treated HCV who presented with acute abdominal pain and was found to have massive splenmogaly secondary to DLBCL. A 65-y.o. female presented with 1-week history of marked sharp left upper quadrant, non-radiating abdominal pain. She had poor appetite, nausea and 3 episodes of coffee-ground emesis over one day. She denied fever and review of systems was otherwise negative. Her history is significant for recently treated hepatitis C with sofosbuvir/velpatasvir a few months prior to this presentation. Physical exam was only positive for tender splenomegaly. CT abdomen showed massive splenomegaly with multiple new splenic infarcts (image 1). She was noted to have a 3-gram drop in hemoglobin in one month, so EGD was done (image 2,3). Her HCV RNA was undetectable by PCR. BCR/ABL and JAK2 mutations, as well as EBV and CMV serology were all negative. Peripheral blood showed mature B-cells. Lymph node biopsy and bone marrow biopsy were diagnostic for DLBCL. Patient was started on chemotherapy with RCHOP regimen with good response. Tumors of the spleen are rare and are classified into benign and malignant. Primary malignancies are extremely rare, with lymphoma being the most common splenic malignancy. Splenic lymphomas are heterogeneous. They may be primary, arising from the spleen, or secondary, involving the spleen as part of systemic dissemination of a lymphoma that arises elsewhere. As a general rule, isolated presentation with splenomegaly is more common in indolent lymphoma than in aggressive lymphoma. The association between hepatitis C virus (HCV) infection and malignant lymphoma is well documented. For patients with hepatitis C, who subsequently develop splenomegaly, it is important for clinicians to be suspicious for splenic DLBCL and not blame HCV as the only cause of splenomegaly especially if it develops after treating HCV. This case is unique as it presents a very rare initial presentation of DLBCL, which is splenomegaly due to splenic involvement without having any other symptoms or signs of systemic involvement. Physicians should be made aware of this rare presentation to avoid delay in diagnosis that can adversely affect patient morbidity and mortality.1925_A Figure 1. CT abdomen shows massive splenomegaly, with multiple infarcts1925_B Figure 2. EGD shows gastric polyp1925_C Figure 3. EGD shows non-bleeding angioectasia