Hypereosinophilic Syndrome Presenting as Intussusception in Adults

Correspondence to Kwang Hyun Kim, M.D. Department of Internal Medicine, Hallym Hospital, 722 Jangje-ro, Gyeyang-gu, Incheon 407-060, Korea Tel: +82-32-540-9114, Fax: +82-32-545-1844, E-mail: kaizersoze@daum.net Copyrightc 2014 The Korean Association of Internal Medicine This is an Open Access article distributed under the terms of the Creative Commons Attribution Non Commercial License (http://creativecommons.org/licenses/by nc/3.0/) which permits unrestricted noncommercial use, distribution, and reproduction in any medium, provided the original work is properly cited. 성인에서 장중첩증으로 발현된 과호산구 증후군 1예


INTRODUCTION
Hypereosinophilic syndrome (HES) is characterized by persistent eosinophilia associated with damage to multiple organs.
Any organ system may be involved in a patient with HES.The heart, central nervous system, skin, and respiratory tract are most commonly involved, whereas HES with predominantly gastrointestinal symptoms is very rare [1].Thus far, only a few cases of HES with gastrointestinal involvement in the form of eosinophilic colitis have been reported [2,3].HES presenting as intussusception in adults has not been reported.We describe such a case of HES presenting as intussusception that was treated with steroids.

CASE REPORT
A 19-year-old male was admitted to our hospital with abdominal pain, nausea, and vomiting for 3 days.He had no his-  (Fig. 2C), and multiple irregular hypoattenuated lesions in both lobes of the liver (Fig. 2D), suggesting multiple eosinophilic liver abscesses.High-resolution chest CT showed a few small nodules surrounded by ground-glass opacities in both lungs, suggesting eosinophilic pneumonitis (Fig. 2E).Other investigations were performed to assess the presence of eosinophil infiltration in other organs.However, the echocardiography findings were unremarkable.Thereafter, gas passage was confirmed during 2 days of fasting, and we performed esophagogastroduodenoscopy and colonoscopy to investigate the esophagus, stomach, duodenal bulb, second portion of the terminal ileum, colon, and rectum.Although endoscopy showed normal mucosa, we performed multiple endoscopic biopsies.A histological examination revealed few mucosal and intraepithelial eosinophils (< 20/highpower field).These findings were not sufficient to diagnose eosinophilic enteritis.
Although we were unable to confirm long-standing eosinophilia, the patient was finally diagnosed with HES based on the presence of idiopathic hypereosinophilia and multiple organ involvement.Intravenous corticosteroid therapy (prednisolone, 60 mg/day) was initiated for the HES with an intestinal obstruction due to intussusception.A marked reduction in abdominal pain was noted after 2 days of treatment.A follow-up abdominal CT after 7 days of intravenous corticosteroid therapy revealed a significant reduction in distal duodenal and jejunal wall edema, fluid collection in the pelvis, multiple ground glass opacities in the lungs, and liver abscesses as well as disappearance of the jejunojejunal intussusception (Fig. 3).The eosinophil count was 434/mm 3 , which was within the normal range.The patient was discharged, and the steroid dose was tapered slowly.

DISCUSSION
Intussusception in adults is a rare condition, accounting for 1-5% cases of bowel obstruction.It is usually primary and benign in children.However, almost 90% of intussusception cases in adults are secondary to a pathological condition [4].Approx- imately 70-90% of intussusception cases in adults require treatment.Surgical resection is the most prevalent treatment [5].
Eosinophilic enteritis has been described as a rare cause of intussusception in two case reports [6,7]; however, HES presenting as intussusception has not been reported.
Eosinophilic disease of the gastrointestinal tract is poorly understood, and there are many probable differential diagnoses.
The possibility of HES should be considered because it can involve multiple organs and has a progressive and potentially fatal course [8].HES is defined by the following: (1) persistently elevated eosinophil count (> 1,500/mm 3 ) for at least 6 months; (2) eosinophilia with no recognizable cause such as parasitic infestation, cancer, or allergic condition; and (3) symptoms of eosinophilia-mediated organ dysfunction.However, a shorter period of hypereosinophilia with symptoms requiring eosinophillowering therapy is also sufficient for diagnosis [9].In the present case, early initiation of systemic corticosteroid therapy resulted in a marked response in a patient with HES presenting as intussusception, and thus obviated the need for surgery.
The clinical manifestations of eosinophilic disease of the gastrointestinal tract vary according to the site and depth of eosinophilic infiltration on the bowel wall.Klein et al. [10] classified this condition into three forms based on the bowel wall layer that is predominantly involved.Symptoms such as diarrhea, hematochezia, and cramping abdominal pain usually occur in the mucosal type.In the muscular type, thickening of the muscular layer narrows the intestinal lumen and can lead to intestinal obstruction or perforation.Ascites may develop if the subserosal layer is involved.In the present case, endoscopic findings and random biopsies did not suggest eosinophilic infiltration.However, we suspected outer layer (muscular or subserosal) involvement due to the presence of intussusception and fluid collection in the pelvic cavity, as noted on abdominal CT.
The present case is the first known case of HES presenting as intussusception in a 19-year-old male.The patient was successfully treated with steroids, and surgery was not necessary.

-Figure 2 .
Figure 2. High-resolution abdominal and chest computed tomography.(A) The target sign is seen in the jejunum, indicating a jejunojejunal intussusception (white arrow).(B) Diffuse wall edema is noted in the distal duodenal and jejunum (black arrow).(C) A small volume of fluid collected in the pelvis.(D) Multiple irregular hypoattenuated lesions are noted in both liver lobes, suggesting multiple eosinophilic liver abscesses.(E) A few small nodules surrounded by ground-glass opacities are seen in both lungs, suggesting eosinophilic pneumonitis (arrowhead).