The patient was a 19-year-old male with a case of thalassemia intermedia who was admitted with severe left upper quadrant pain and was suspected for splenic crisis. At the time of admission, the patient’s vital signs were 37.6°C temperature, 78 beats per minute heart rate, 20 breaths per minute respiration rate and 128/75 mm Hg blood pressure.
Past medical history revealed thalassemia intermedia with a history of right upper abdomen pain for the past 6 months and a loss of appetite due to chronic cholecystitis. An allergy to fava beans, known as favism, was also apparent. Medications consisted of folic acid tablets, periodic pneumococcal vaccinations, and at rare times a blood transfusion. The patient had not recently received courses of antibiotics or steroids and he denied any illicit drug or herbal supplement usage. He did not drink alcohol or smoke.
A physical examination was pertinent for his left upper quadrant tenderness and the enlargement of his spleen (Palpable 3 cm below the costal margin). Right upper quadrant tenderness was not detected during the examination and the Murphy sign was also not identified.
The initial laboratory tests are outlined in Table 1, which notes a mildly decreased Hb level, no eosinophilia, and an elevated bilirubin level, which results in a hemolytic anemia diagnosis. An ultrasound & CT scan of the abdomen demonstrated splenomegaly, increased spleen parenchymal echogenicity, and multiple gallstones.
The patient rejected additional imaging studies, such as Magnetic Resonance Imaging (MRI), due to his claustrophobia.
The patient was prescribed cephalosporin and penicillin during his hospital stay and underwent an open splenectomy due to his splenic crisis and a cholecystectomy due to his chronic cholecystitis. No intra or post- operative complications were encountered. The patient was discharged two days after surgery.
Histopathology findings showed a congested splenic tissue (Figure 1) and eosinophilic cholecystitis (Figure 2). On gross examination of specimen, the gallbladder was pear-shaped and measured 12 x7x4 cm and on opening gallbladder the maximal wall thickness was 0.3 cm. The internal surface had a velvety green mucosa which contained multiple stones with the maximum measurement of 1.5 cm. Microscopic views revealed a marked eosinophilic infiltration with few lymphoplasma cell infiltrations, which confirmed the diagnosis of EC. There were no evidence of epithelial dysplasia, parasitic infestation, or identified malignancy.

Table 1. Laboratory test results

Figure 1. A. Congestive splenic tissue (40x, H&E stain) B. Eosinophilic cholecystitis (40x, H&E stain)

Discussion

EC is an infrequent inflammatory condition of the gallbladder with a prevalence of 0.25% to 6.4% in all patients with cholecystitis (5,8). The mean age of presentation occurs at about 40 years with no detected incidences in a specific gender or race [9,10].
Clinically, EC cannot be distinguished from causes of cholecystitis and its diagnosis is based on classical symptoms of cholecystitis such as right upper quadrant (RUQ) pain and Murphy^’s sign (patient firmly places his or her hand at the costal margin in the RUQ and with deep inspiration will have extreme pain). Thus, the diagnosis is based on the histopathological findings of a cholecystectomy specimen.
Although no definitive etiology can be drawn for EC, it has been reported as idiopathic or in combination with autoimmune reactions, allergies, hypereosinophilic syndromes (HES), parasites (Hepatic echinococcosis), eosinophilic gastroenteritis, and hypersensitivity to drugs and herbal medicines (5,6,11). In histopathology, if cellular infiltrate consists of 90% eosinophils termed as EC, but when the infiltrate consists of 50% to 75% eosinophils along with other inflammatory cells in the gallbladder wall, they are classified as Lymphoeosinophilic cholecystitis (12,13).
Calculi are associated in 40% of cases of both eosinophilic and Lymphoeosinophilic varieties (14). EC prevalence in acalculous cholecystitis is three times more than that of cholelithiasis (2). In literature acalculous cholecystitis is reported in obstructive jaundice which is more common in men (12).
In a conducted study of 7,494 patients who underwent cholecystectomies in a 14 year period, the prevalence of EC was 0.16%, which is 1 case for every 625 cholecystectomies performed. All of them were idiopathic and had concomitant gallstones (15). After evaluating clinical examinations, history and investigations of this case report, the only noteworthy causes of EC were an allergic history, favism, and cephalosporin medications during hospital stay, which could be considered as predisposing etiologies.
The definitive treatment of EC is surgical cholecystectomy; however, medical management such as steroids may be useful in EC associated patients of eosinophilic gastroenteritis or HES, depending on the severity of disease (12,16). In our patient, cholecystectomy led to full recovery. The patient was also periodically monitored at the outpatient department and passed an uneventful postop course.

 

Conclusion

EC is an infrequent condition with unknown etiology. Gastroenterologists, pathologists, and surgeons need to consider EV because if a causative factor is discovered, follow up and treatment may lead to diagnosis of an important disease. The diagnosis of eosinophilic cholecystitis is based on histopathologic findings because it has no specific clinical or laboratory feature and cholecystectomy is the definitive treatment. As seen in our case, EC was associated with favism and thalassemia intermediate which in accordance to the findings of medical texts, no such case has been previously reported.

 

Acknowledgements

The authors would like to express their gratitude to the Clinical Research and Development Unit of Ayatollah Rouhani Hospital in Babol, Iran.

 

Conflicts of Interest

Authors declared no conflict of interests.