A Hypereosinophilic Syndrome Presenting as Eosinophilic Colitis
A 56-year-old man visited the hospital with complaints of more than 30 bloody diarrheas per day, combined with lower abdominal pain and febrile sensation for 5 days. GI symptom of alternating constipation and diarrhea had persisted for past 3 months. He also had respiratory symptom of chronic cough. He was taking no medications, including herbs and drugs, and had no history of food or drug allergies.\nOn admission, his blood pressure was 120/80 mm Hg, pulse rate 85/min, respiratory rate 20/min, and body temperature 37.0℃. Physical examinations showed moderate tenderness of the lower abdomen. White blood cell count was 21,040/mm3 with markedly increased eosinophils (11,151/mm3, 53%). Other serum chemistries were normal. Negative test results were obtained for viral markers (hepatitis A, B, C, adenovirus, and human immunodeficiency virus), serum antibodies to parasites, and autoimmune antibodies (anti-nuclear, anti-dsDNA, and anti-neutrophilic cytoplasmic antibodies), tumor markers (alpha-fetoprotein, carbohydrate antigen 19-9, and carcinoembryonic antigen), and rheumatoid factor. No pathogens were detected in a microbiological blood culture, and stool examinations for parasites and protozoa were negative. Simple chest and abdomen X-ray studies were normal.\nThe colonoscopy revealed multifocally erythematous and edematous mucosal changes with disappearance of normal vascular markings and easy-touch bleeding throughout the entire colon, including the rectum (). There was intense infiltration of the mucosa by eosinophils in the specimens obtained from colonic mucosa (). Although esophagogastroduodenoscopy showed only erythematous change in the antrum of the stomach (), microscopic findings of specimens from random biopsies of the stomach and duodenal mucosa were similar to those of the colon. Chest computed tomography (CT) for evaluation of the chronic cough showed multiple nodular opacities on the both lung, suggesting eosinophilic pneumonitis (). Abdominal CT suggested a diagnosis of multiple eosinophilic liver abscesses, which were multifocal, patchy enhancing lesions on the right lobe of the liver in the arterial phase and ill-defined low-density lesions at the same site in the portal phase (). Nothing remarkable was seen on echocardiography. Bone marrow aspiration and biopsy studies revealed eosinophilic hyperplasia with normal maturation. No blast or FIP1L1/platelet-derived growth factor receptor-alpha (PDGFRA) fusion transcripts were detected, and the results of fluorescence in situ hybridization for BCR/ABL were normal. A chromosomal analysis showed no apparent cytogenetic abnormalities.\nThe patient was finally diagnosed with HES with involvement of the colon, stomach, duodenum, liver, and lung. Because hypereosinohilia was present for 3 months before his HES diagnosis, hypereosinophic period was shorter than the diagnostic criteria for HES. However, oral corticosteroid therapy (prednisolone, 60 mg/day) was started for HES with severe symptoms caused by multiple organ involvement. Three days later, symptoms such as abdominal pain, bloody diarrhea, and cough were improved dramatically. The patient was discharged, and the steroid dose was slowly tapered. Follow-up blood tests in the outpatient department demonstrated normalization of eosinophil count.