Catastrophic Hypereosinophilic Syndrome with Eosinophilic Cystitis: A Rare Association and Challenges in Treatment

1Case Western Reserve University/St. Vincent Charity Medical Center, Cleveland, OH
2Cleveland Clinic, Cleveland, OH
3Case Western Reserve University/St. Vincent Charity Medical Center, Cleveland, OH
4Case Western Reserve University/St. Vincent Charity Medical Center, Cleveland, OH
5Cleveland Clinic, Cleveland, OH

Meeting: Hospital Medicine 2013, May 16-19, National Harbor, Md.

Abstract number: 430

Case Presentation:

A 56‐year old previously healthy woman presented with 1‐month history of a pruritic ulcerated rash in both lower extremities associated with dysuria and intermittent chest pains. On presentation, the patient was hypotensive with a systolic blood pressure of 56 mm Hg. Physical exam disclosed a displaced point of maximal impulse and audible 3/6 diastolic murmur and noticed macular ulcerated rash in both legs. Initial labs showed WBC 15,000, remarkable eosinophilia 30%, and elevated cardiac enzymes. Echocardiography demonstrated impaired left ventricular systolic function with EF of 45%. Despite antibiotics, intravenous fluids, and all the resuscitative measures, the patient remained critically ill. Later, she developed gross hematuria and developed renal failure. CT abdomen pelvis showed a 5 × 4 cm mass at the dome of the bladder with marked bladder wall thickening. Cystoscopy revealed a solid mass invading the bladder wall and the entire mucosa appeared hemorrhagic and congested. Bladder mass biopsy showed infiltration of abundant eosinophils from the submucosa to the muscular layer. The diagnosis of hypereosinophilic syndrome (HES) with eosinophilic cystitis was made. The patient was started on prednisone 1 mg/kg per day and hydroxyurea. The patient's condition started to improve gradually and the rash faded away as well as the peripheral eosinophilia resolved.

Discussion:

Hypereosinophilic syndrome (HES) is a rare disorder characterized by the overproduction of eosinophils in the bone marrow with persistent peripheral eosinophilia, associated with organ damage by the release of eosinophilic mediators. It is defined by three diagnostic criteria: persistent eosinophilia >1.5 × 109/L with organ damage or dysfunction and exclusion of secondary causes of eosinophilia such as parasitic infection or allergic reactions. The aberrant cytokine production such as IL‐4 and IL‐5 can promote eosinophilic proliferation and activation resulting in HES that leads to organ damage. Eosinophilic cystitis is a rare condition characterized histologically by eosinophilic infiltration of the submucosa and muscular layer. Radiographic findings may show variable thickening of the bladder wall, ranging from diffuse thickening to mass formation resemble invasive bladder tumor. Diagnosis can be made by histological examination of a biopsy specimen of the bladder.

Conclusions:

This case increases the practitioners' general awareness of HES syndrome. It is characterized by sustained overproduction of eosinophils, in which eosinophilic infiltration cause damage to multiple organs. Frequent follow‐up surveillance is mandatory in patient with HES. Although bladder involvement is very rare, cutaneous lesions are common and may be the presenting sign of more serious condition. Systematic approach and careful examination for the multiorgan involvement in patient with this condition is crucial as early detection and prompt treatment result in better prognosis.

To cite this abstract:

Dinary B, Alraies M, Hader I, Ravakhah K, Shaheen K. Catastrophic Hypereosinophilic Syndrome with Eosinophilic Cystitis: A Rare Association and Challenges in Treatment. Abstract published at Hospital Medicine 2013, May 16-19, National Harbor, Md. Abstract 430. Journal of Hospital Medicine. 2013; 8 (suppl 2). https://www.shmabstracts.com/abstract/catastrophic-hypereosinophilic-syndrome-with-eosinophilic-cystitis-a-rare-association-and-challenges-in-treatment/. Accessed July 21, 2019.

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