Zilan Lin, MD, Wei Tang, MD, Brad Dworkin, MD, Rathnamitreyee Vegunta, MD, Amir Steinberg, MD, Sidra Zaheer, MD, Fouzia Shakil, MBBS, PhD, Beth Schorr-Lesnick, MD Westchester Medical Center, Valhalla, NY
Introduction: Extranodal marginal zone lymphoma of the mucosa-associated lymphoid tissue (MALT) most frequently involves gastrointestinal (GI) tract. Immunoproliferative small intestinal disease (IPSID), also called alpha heavy chain disease (αHCD), is a variant of MALT lymphoma that secretes alpha heavy chains. The pathogenesis of MALT lymphoma is linked to clonal B cell expansion from chronic immune stimulation by infectious agents. We report a rare case of GI αHCD with five concomitant pathogens identified on a GI multiplex real-time polymerase chain reaction panel, featured by persistent Campylobacter jejuni (C jejuni) colonization and refractory giardiasis.
Case Description/Methods: A 34-year-old man with three-month history of lower abdominal pain, intermittent bloody diarrhea and weight loss was admitted following outside colonoscopy, which revealed congested and nodular terminal ileum and serum electrophoresis detection of a monoclonal alpha heavy chain suspicious for αHCD. Repeat scopes (Fig.1) with biopsies at our facility confirmed intestinal monocytoid lymphocytes infiltration positive for CD20, MUM-1, and IgA featuring αHCD (Fig.2), and Helicobacter pylori (H pylori) infection. A GI multiplex was collected and positive for C jejuni, Giardia lamblia, Enteroaggregative and Enteropathogenic Escherichia coli (EAEC, EPEC), and Cryptosporidium; results were confirmed on a repeat test 48 hours later. He was treated with antibiotics and started on Bendamustine plus Rituximab (BR) for αHCD. His GI symptoms improved and H pylori was eradicated. However, similar symptoms recurred three times within nine months, and GI multiplex tests showed persistent colonization of C jejuni and refractory Giardia despite treatment with different antibiotics for prolonged courses (Fig.3). In addition, repeat endoscopy after six cycles of BR showed relapse αHCD, prompting escalation of therapy to ibrutinib.
Discussion: To our knowledge, this is the first case of GI αHCD with five pathogens concomitantly identified on GI multiplex. Associations between H pylori and gastric MALT lymphoma or C jejuni and αHCD have been established. We suspected that persistence of C jejuni colonization and giardiasis despite extensive antibiotic treatment correlated with the progression of lymphoma, contributed to failure of BR therapy, and portended a poor long-term prognosis. Further studies are needed to support the identification and eradication of all infectious agents with lymphomagenic capability as part of routine care for αHCD.
Figure: Figure 1 Esophagogastroduodenoscopy and Colonoscopy Findings. A. Congestion, erythema and nodularity in the anterior duodenal bulb. B. Edema, erythema and nodularity in the antrum and stomach body compatible with infiltrative disease. C. Congestion, erythema and nodularity in the second portion of duodenum. D. Edema, congestion and nodularity in the terminal ileum. Figure 2 Histopathological Findings of Terminal Ileum Biopsy. A. High magnification (20x) H&E section of terminal ileum showing infiltration of the mucosa by sheets of atypical monocytoid lymphocytes. Destruction of crypts with lymphoepithelial lesion is also identified. B. Atypical cells showing positivity for CD20. C. Atypical cells showing positivity for MUM-1. D. Atypical cells showing positivity for IgA. Figure 3 Timeline of Disease Course with Treatment. Abbreviations: αHCD, alpha heavy chain disease; GI, gastrointestinal; EGD, Esophagogastroduodenoscopy; C jejuni, Campylobacter jejuni; EBV, Epstein-Barr virus; EAEC, Enteroaggregative E coli; EPEC, Enteropathogenic Escherichia coli; H pylori, Helicobacter pylori; (+), Positive; (-) Negative.
Disclosures:
Zilan Lin indicated no relevant financial relationships.
Wei Tang indicated no relevant financial relationships.
Brad Dworkin indicated no relevant financial relationships.
Rathnamitreyee Vegunta indicated no relevant financial relationships.
Amir Steinberg: Jazz Pharmaceuticals – Speakers Bureau.
Sidra Zaheer indicated no relevant financial relationships.
Fouzia Shakil indicated no relevant financial relationships.
Beth Schorr-Lesnick indicated no relevant financial relationships.
Zilan Lin, MD, Wei Tang, MD, Brad Dworkin, MD, Rathnamitreyee Vegunta, MD, Amir Steinberg, MD, Sidra Zaheer, MD, Fouzia Shakil, MBBS, PhD, Beth Schorr-Lesnick, MD. P1333 - A Case of Gastrointestinal Alpha Heavy Chain Disease With Persistent Campylobacter jejuni Colonization and Refractory Giardiasis, ACG 2023 Annual Scientific Meeting Abstracts. Vancouver, BC, Canada: American College of Gastroenterology.
