An Atypical Association of Hepatitis C Infection With Extra Nodal Marginal Zone Lymphoma

Abstract

Marginal Zone lymphomas (MZLs),uncommon form of B cell Non Hodgkin lymphomas, are classified into 3 groups based on the tissue involved: Extra Nodal MZL or Mucosa associated Lymphatic tissue lymphoma (MALT lymphoma),Splenic MZL & Nodal MZL. We present a rare case of extra nodal MZL of the ocular adnexa due to hepatitis C Infection. The paucity of evidence of this association makes our case unique, offering a different therapeutic outcome with treatment of hepatitis C infection in conjunction with traditional chemotherapy. A 68 year old male presented with 3 week history of right eye pain, swelling, blurry vision and diplopia. He was an active cocaine user and drank alcohol socially. He had unintentional weight loss of 20 pounds in 2 months. He had no blood transfusions prior to 1980 and no tattoos. Abnormal physical exam findings included proptosis of the right eye, bilateral eye lid swelling, non-tender axillary and inguinal lymphadenopathy. He tested positive for hepatitis C infection with an elevated viral load, PCR with active virus, 1a genotype, F1-2 fibrosis. Hepatitis B core IgG antibody testing was positive with a negative surface antigen/antibody and HBV DNA.CT head/orbits showed enlargement of the right superior rectus muscle with resultant proptosis of the right globe. CT chest/abdomen/pelvis revealed mediastinal and axillary lymphadenopathy. Subsequent excision biopsy of the right axillary lymph node established the diagnosis of MZL, supported by bone marrow biopsy and tandem flow cytometry .His symptoms improved with a 5 day course of Prednisone 80 mg daily. He was discharged with a plan to follow up with oncology and gastroenterology for initiation of chemotherapy and treatment of hepatitis C infection. MALT Lymphoma develops in sites that are normally without any lymphoid tissues such as the GI tract, salivary glands and eyes due to long standing antigen stimulation either in the form of an autoimmune disease(Salivary gland lymphoma & Sjogren's syndrome) or a chronic infectious process (MALT lymphoma & H.pylori). Growing evidence supports the association of hepatitis C virus with B cell NHL such as diffuse large B cell lymphoma, small lymphocytic lymphoma, splenic and nodal MZLs. Extra nodal MZLs such as orbital MZL are not associated with hepatitis C. Hepatitis C virus was the potential cause of the patient's orbital MZL and it needs to be further validated in future research as it has significant therapeutic implications.Figure: Proptosis of the right eye and bilateral eye lid swelling.Figure: CT head/orbits with mass like enlargement of the right superior rectus muscle with resultant proptosis of the right globe.Figure: Excision biopsy of the right axillary lymph node showing histopathology features consistent with diagnosis of marginal zone lymphoma.