Hypereosinophilic Syndrome Presenting With Gastrointestinal Complaints

Abstract

A 33 year old woman with history of rheumatoid arthritis, systemic lupus erythematosus, Sjogren's syndrome and hypereosinophilic syndrome presented to the hospital with six weeks of cramping abdominal pain, vomiting and alternating non-bloody diarrhea and constipation. She denied fevers, chills, night sweats or recent travel. She had a recent hospitalization for similar complaints; stool cultures and Clostridium difficile PCR were negative, and a colonoscopy showed lymphoid aggregates in the lamina propria of the terminal ileum and colon. Upper endoscopy suggested gastroparesis and duodenitis. She was treated there with ciprofloxacin, metronidazole, and colchicine, and was discharged. Her symptoms persisted and so she presented to our hospital. A CT scan on admission showed large volume ascites and jejunitis and ileitis. Paracentesis yielded 9750 white cells, with 98% eosinophils. Labs revealed peripheral eosinophilia of 16.4%. Stool cultures, Clostridium difficile PCR and ova and parasites were all negative. A bone marrow biopsy was performed and had 20% eosinophilia without blasts. She continued to experience abdominal pain and alternating bowel habits. She was started on prednisone 1mg/kg/day with dramatic improvement of symptoms and peripheral eosinophilia. Hypereosinophilic syndrome presenting with gastrointestinal symptoms is an unusual presentation. Criteria to make the diagnosis require persistent peripheral eosinophilia greater than 1500/mmˆ3 for more than 6 months, no other suspected etiologies, and signs and symptoms of organ involvement [1, 2]. We were able to rule out other potential causes of gastrointestinal eosinophilia including infection, inflammatory and oncologic conditions. Biopsies had also revealed the absence of eosinophilic infiltrates, to suggest that this was not a diagnosis of eosinophilic enteritis, but hypereosinophilic syndrome [3]. In our case, she was noted to have a predominance of eosinophils in her ascites fluid; the presence of eosinophilic ascites has been only rarely reported in the literature in association with hypereosinophilic syndrome [4]. The mainstay treatment for hypereosinophilic syndrome is steroids [2, 5]. Our patient was ultimately given steroids, and had significant improvement of her symptoms. When presenting with gastrointestinal symptoms and evidence of eosinophilia, one must keep in mind the diagnosis of hypereosinophilic syndrome so the patient can receive the appropriate treatment.