1984 Helicobacter pylori-Negative MALT Lymphoma Presenting as Massive Recurrent Gastrointestinal Hemorrhage

Abstract

INTRODUCTION: Extranodal marginal zone lymphoma or low-grade B cell lymphoma of mucosa-associated lymphoid tissue (MALT), an uncommon type of non-Hodgkin lymphoma (NHL), accounts for 5–10% of NHL in the US (18.3 cases per 1 million person-years1). It usually presents as gastro-esophageal reflux, nausea, anorexia, weight loss or occult GI bleeding2. Massive GI bleeding is unusual and rare. CASE DESCRIPTION/METHODS: Here we present a 68-year-old woman who presented with weakness and syncope after large volume hematemesis. Physical exam showed tachycardia, hypotension and conjunctival pallor. Her hemoglobin and hematocrit on admission were 8.5 g/dL and 25.6 %. She was managed with IV fluids, packed RBC transfusion and Pantoprazole and Octreotide infusions. An EGD within 24 hours showed erythematous, friable, spontaneously oozing mucosa with neovascularization in gastric fundus which was treated with bipolar cautery. A biopsy was not obtained due to recent bleeding. She was discharged on oral Pantoprazole. Two days later she was readmitted due to severe recurrent hematemesis with similar findings on repeat EGD. Bleeding was controlled with APC and biopsies were obtained from the gastric fundus. Due to the recurrent bleeding, an angiogram was performed. Active bleeding could not be seen. Prophylactically, left gastric artery was embolized. Biopsies revealed low-grade B cell MALT lymphoma with negative H.pylori. She was treated with 20 fractions of radiotherapy with the intention to cure. She did not experience further episodes of bleeding and has remained in remission since. DISCUSSION: Studies show the indolent nature of GI MALT lymphoma3, but our case illustrates the life-threatening presentation of a rare low-grade B cell MALT lymphoma as massive and recurrent hematemesis with negative underlying H.pylori infection or autoimmune process45. H.pylori positive localized MALT lymphoma is treated with the eradication of infection. H pylori negative, localized and recurrent disease is treated with radiotherapy6. Chemotherapy and/or immunotherapy is given when patients fail to respond to radiotherapy or develop metastasis. Long term endoscopic and laboratory follow up is needed as gastric MALT lymphoma is associated with a significant risk of gastric adenocarcinoma8 and increased incidence of gastric cancer and residual NHL. Our case emphasizes the importance of early diagnosis and timely intensive radiotherapy of a localized but aggressive gastric MALT lymphoma which induced remission and cessation of GI bleeding.