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Saudi Journal of Kidney Diseases and Transplantation
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Table of Contents   
LETTER TO THE EDITOR  
Year : 2014  |  Volume : 25  |  Issue : 6  |  Page : 1301-1303
Eosinophilic cystitis and idiopathic hypereosinophilic syndrome in an eight-year-old girl


1 Shiraz Nephrology Urology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran
2 Shiraz University of Medical Sciences, Shiraz, Iran

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Date of Web Publication10-Nov-2014
 

How to cite this article:
Derakhshan D, Ilkhanipoor H, Derakhshan A. Eosinophilic cystitis and idiopathic hypereosinophilic syndrome in an eight-year-old girl. Saudi J Kidney Dis Transpl 2014;25:1301-3

How to cite this URL:
Derakhshan D, Ilkhanipoor H, Derakhshan A. Eosinophilic cystitis and idiopathic hypereosinophilic syndrome in an eight-year-old girl. Saudi J Kidney Dis Transpl [serial online] 2014 [cited 2019 Jul 17];25:1301-3. Available from: http://www.sjkdt.org/text.asp?2014/25/6/1301/144272
To the Editor,

Eosinophilic cystitis (EC) as an initial symp­tom of the idiopathic hypereosinophilic syn­drome is a very rare clinical entity in the pediatric population. [1],[2],[3] It is often misdiagnosed with urinary tract infection, bladder tumor, and parasitic infection. [1],[4] Kindall and Nickels, in 1949, stated that eliminating food allergens from a diet can improve the signs and symp­toms of cystitis in patients with identified eosinophilic leukocytes in the urine or bladder wall. [5] Since then, 31 cases of EC have been reported in the literature.

The incidence of EC is equal in both sexes, but in children, boys are affected more than girls. [5] The most common predisposing factors include regulatory disorder of the immune system, parasitosis, food and drug allergies, malignancy, tuberculosis, and chronic vesicle irritation. [1] The most common presenting symp­toms include frequency, dysuria, gross or microscopic hematuria, suprapubic and abdomi­nal pain, and urinary retention. [2],[5],[6] Ultrasound, computed tomography, and a voiding cystourethrogram show diffuse thickening of the bladder wall with a filling defect. [6] Diagnosis is confirmed by transurethral cystoscopy and submucosal punch biopsy. [2]

On account of the limited number of cases and inadequate clinical experience, standardized therapeutic regimens do not exist. [1],[3] Any suspected allergen must be removed. [4] In a pooled analysis of 135 cases, the most successful treatment regimen was transurethral resection of the lesion combined with corticosteroids, anti-histaminics, and antibiotics. [5] However, in the hypereosinophilic syndrome, the goal of treatment is reducing the circulating eosinophils to avoid organ involvement. [3] Most patients are cured, but recurrence is a frequent finding. [5] As we could not find a similar report in our region, we are presenting our experience in managing a child with EC.

Our patient is an eight-year-old girl, who pre­sented with a history of dysuria, frequency, and suprapubic pain of a one-week duration. There was no history of fever. The patient did not have any history of prior urinary tract infection. Also, she did not have any history of asthma or allergic disease. An initial workup revealed normal urine analysis and a negative urine cul­ture. A complete blood count (CBC) showed significant eosinophilia (34%), with an abso­lute esosinophil count of 4700. Other indices of CBC were normal for the patient's age and sex. The blood urea nitrogen and creatinine were 15 mg/dL and 0.7 mg/dL, respectively. The chest x-ray and echocardiography were normal. investigations for parasitic infections, including, toxocariasis, Echinococcus, Strongyloides stercoralis, and schistosomiasis, which were all negative. Bone marrow examination revealed 28% eosinophils, but otherwise, it was normal. A skin test with five units of purified protein was negative. Ultrasound examination of the genitourinary tract showed a significantly increased bladder wall thickness to about 15 mm and marked mucosal irregularity, sugges­tive of severe cystitis. The patient underwent cystoscopy that showed a tight urethra and moderate trabeculation of the bladder wall with collection of debris. Bladder wall biopsy showed bladder mucosal fragments with a benign urothelial epithelium and moderate eosinophilic infiltrates consistent with EC. A diagnosis of the hypereosinophilic syndrome was made on the basis of the presence of hypereosinophilia with no other identifiable cause or organ involvement, according to Chusid. [7]

Treatment with prednisolone was started in a dose of 1 mg/kg/day. The symptoms diminished within one week of steroid treatment. Ultra­sound examination of the bladder revealed a normal appearance with a wall thickness of 2.5 mm. The CBC had also normalized. The steroid was continued at the same dose for two weeks and was tapered to alternate-day therapy, and subsequently discontinued. Unfortunately, the patient's symptoms recurred after discontinuation of the steroid therapy, and at this time, the eosinophil count was 46%. Another course of steroids resulted in a dramatic response and the steroids were gradually tapered and the patient is on 2.5 mg prednisolone for six months at the time of reporting, with the eosinophil count being 4%, and she is without any urinary symptoms.

EC is a rare clinical disease in children cha­racterized by eosinophilic infiltration of the bladder wall with variable presentation and outcome. It may occur in all age groups. In children, there is a slight male predominance, although our case was a girl. [2],[8] Joseph et al. reported a boy with EC, who had oliguria, edema, hypertension, and renal insufficiency. [2] Association of EC with allergy has been proposed in the literature, [2],[4] but the importance of the allergic factors is not obvious. Other associated conditions include trauma, medi­cation, and bacterial, viral, and parasitic infection. [9] Neither allergy nor other associated conditions were found in our patient. Although eosinophilia in the peripheral blood may contribute to the diagnosis, it is an infrequent finding. [8] However, our patient had significant peripheral eosinophilia, which resolved after corticosteroid therapy. Van den Ouden et al did not find any correlation between disease re­currence and peripheral eosinophilia, although they recommended monitoring the treatment with a routine determination of eosinophils in the peripheral blood. [5] Conversely, Sterrett et al believed that a higher blood eosinophil level was associated with disease activity and merited more aggressive treatment. [9]

Definitive diagnosis of the disease is made by transurethral endoscopy and submucosal punch biopsy. [1] Cystoscopy must be performed to ex­clude other possible diagnoses. Similar to other cases in literature, our case was diagnosed by transurethral cystoscopic biopsy and histopathological investigations proved the diag­nosis of EC. We performed bone marrow aspi­ration and biopsy due to the severe eosinophilia to rule out related malignancies.

The natural course of the disease is usually self-limiting in children. [4],[10] Treatment is largely empirical and mainly includes withdrawal of any antigen, if identified. The recommended treatment options for EC involve anti-histaminics and non-steroidal anti-inflammatory drugs as first-line agents and corticosteroids, cyclophosphamide, doxorubicin, cyclosporine, and azathioprine or monteleukast sodium for refractory cases. [9] Other recommended thera­peutic options include hydrodistension, radio­therapy, intravesical silver nitrate, and surgery. [1],[8],[9],[11]

EC may recur in children after cessation of medical therapy. [1],[5] The recurrence rate in a review of 135 cases was estimated to be 28%. Abilov reported a recurrence of the disease during tapering of antihistaminics. [1]

In conclusion, EC is a rare condition in the pediatric age-group with a wide range of clin­ical manifestations. Although not common, it should be considered in patients with lower urinary tract symptoms without evidence of infection or other common causes.


   Acknowledgment Top


The authors wish to thank Professor Mahdi Shirazi (Associate Professor of Urology) for performing cystoscopy and Professor Perikala Vijayananda Kumar (Professor of Pathology) for reporting the pathological findings. We also thank our colleagues in the Hematology Depart­ment for their kind assistance in the manage­ment of our patient.

 
   References Top

1.
Abilov A, Ozcan R, Polat E, Dervi o lu S, Emir H. Rare cause of dysuria: Eosinophilic cystitis. J Pediatr Urol 2013;9:e6-8.  Back to cited text no. 1
    
2.
Galutira PJ, Canonigo BB, Cabansag MR, Bolong DT, Ong RC, Lopez RA. Presenting manifestations of eosinophilic cystitis in two Filipino children. Int Urol Nephrol 2010;42: 557-63.  Back to cited text no. 2
    
3.
Nofal R. Eosinophilic cystitis as symptom of an idiopathic hypereosinophilic syndrome. abstract. Aktuelle Urol 2007;38:148-51.  Back to cited text no. 3
    
4.
Verhagen PC, Nikkels PG, de Jong TP. Eosinophilic cystitis. Arch Dis Child 2001;84:344-6.  Back to cited text no. 4
    
5.
van den Ouden D. Diagnosis and management of eosinophilic cystitis: A pooled analysis of 135 cases. Eur Urol 1997;50:750-4.  Back to cited text no. 5
    
6.
Cai B, Xie H, Xu MS, et al. Eosinophilic cystitis in children: A report of six cases and review of literature. Zhonghua Yi Xue Za Zhi 2003;83:1791-4.  Back to cited text no. 6
    
7.
Chusid MJ, Dale DC, West BC, Wolff SM. The hypereosinophilic syndrome: Analysis of fourteen cases with review of the literature. Medicine (Baltimore) 1975;54:1-27.  Back to cited text no. 7
    
8.
Thompson RH, Dicks D, Kramer SA. Clinical manifestations and functional outcomes in children with eosinophilic cystitis. J Urol 2005; 174:2347-9.  Back to cited text no. 8
    
9.
Sterrett S, Morton J, Perry D, Donovan J. Eosinophilic cystitis: Successful long-term treatment with montelukast sodium. Urology 2006;67: 423.e19-423.e21.  Back to cited text no. 9
    
10.
Hwang EC, Kwon DD, Kim CJ, et al. Eosinophilic cystitis causing spontaneous rupture of the urinary bladder in a child. Int J Urol 2006; 13:449-50.  Back to cited text no. 10
    
11.
Popescu OE, Landas SK, Haas GP. The spec­trum of eosinophilic cystitis in males: Case series and literature review. Arch Pathol Lab Med 2009;133:289-94.  Back to cited text no. 11
    

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Correspondence Address:
Dr. Ali Derakhshan
Shiraz Nephrology Urology Research Center, Shiraz University of Medical Sciences, Shiraz
Iran
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DOI: 10.4103/1319-2442.144272

PMID: 25394455

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