Weill Cornell Medicine - Qatar New York, NY, United States
Omar Mhaimeed, 1, Zachary Sherman, MD2, David Wan, MD3 1Weill Cornell Medicine - Qatar, New York, NY; 2New York Presbyterian - Weill Cornell Medical Center, New York, NY; 3New York Presbyterian Weill Cornell Medicine, New York, NY
Introduction: Hypereosinophilic syndromes (HES) are defined by eosinophilia in peripheral blood and are accompanied by tissue involvement.1 The estimated prevalence is 0.36 to 6.3 per 100,000.2 In a retrospective series of 188 patients, 14% presented with gastrointestinal symptoms3. 17% of patients with HES have the lymphocytic variant (LHES) which is characterized by aberrant monoclonal T cells on immunophenotypic analysis.3,4
Case Description/Methods: A 69-year-old African American man with SLE and ESRD presented with abdominal pain and diarrhea. Two months prior, he was diagnosed with C. difficile colitis that resolved with vancomycin. He then began having severe epigastric pain, early satiety, a 20-pound weight loss, and his diarrhea recurred. He was found to have a hemoglobin of 6.4 and an elevated absolute eosinophil count of 1.35 x 103 u/L. Viral and parasitic work-up were unremarkable. Endoscopy was performed and showed non-bleeding gastric ulcers, and colonoscopy showed erythematous mucosa in the descending colon. Pathology revealed chronic gastritis along with chronic enteritis in the duodenum. Pan-colonic biopsies showed chronic colitis and lymphohistiocytic infiltrates in the lamina propria. These inflammatory cells consisted of mostly atypical, activated lymphocytes and histiocytes. Immunostaining revealed the lymphocytes to be CD3+ and the histiocytes to be CD163+. He was started on high dose steroids which led to improvement in his symptoms. Bone marrow biopsy was done to investigate the hypo-proliferative anemia and revealed eosinophils accounting for 18% of the cellularity and an aberrant clonal population of T-cells. Flow cytometry was consistent with a T-cell lymphoproliferative disorder. Mepolizumab was added as his steroids were tapered, and his symptoms went into remission.
Discussion: LHES manifests with a spectrum of GI findings including abdominal pain, diarrhea, vomiting, and hepatic involvement.5 Endoscopic findings of HES described in case reports include gastritis and ulcers. Certain laboratory findings suggesting LHES include eosinophilia, elevated IgE, aberrant T-cell immunophenotypes, and molecular evidence suggesting T-cell clonality on biopsies obtained from bone marrow or other tissues.6,7 The course of LHES can be complicated by neoplasms, most often lymphomas.4 The first-line treatment for LHES is systemic steroids, and mepolizumab is the only effective steroid-sparing agent as demonstrated in a randomized clinical trial. 3,8,9
Figure: Figure 1 A. Endoscopic photograph showing erythematous antral mucosa with non-bleeding ulcers. B. Congested duodenal mucosa and erythematous duodenopathy. C. Erythematous mucosa in the descending colon.
Omar Mhaimeed indicated no relevant financial relationships.
Zachary Sherman indicated no relevant financial relationships.
David Wan indicated no relevant financial relationships.
Omar Mhaimeed, 1, Zachary Sherman, MD2, David Wan, MD3. P2279 - Lymphocytic Variant of Hypereosinophilic Syndrome Presenting with Gastroenteritis, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.