Patient report
In December 2017, a 49-year-old man was admitted to the Gastroenterology Department of the People’s Hospital of Nanchuan Chongqing for intermittent lower abdominal pain, intermittent diarrhoea and occasional mucous bloody stools. The first enteroscopy suggested multiple ulcers in the colon. Pathology suggested chronic inflammatory changes. Because ulcerative colitis could not be excluded, 5-aminosalicylic acid was given for 4 weeks, after which Clostridium butyricum supplemented with intestinal probiotics was intermittently administered. In 2018-2019, repeat enteroscopies still suggested multiple ulcers in the colon, and pathology suggested chronic inflammation. During the course of the disease, the patient had no fever or night sweats, consumed a high-fat food without diarrhoea, and did not lose weight. The patient had no history of long-term heavy drinking and no personal or family history of immunodeficiency-related diseases. In May 2020, the patient was again hospitalized due to diarrhoea and mucous and bloody stools.
The routine laboratory test results were as follows: CRP 38.67 mg/L, CA72-4 10.32 U/ml, UA 446 µmol/L. The faecal occult blood test was positive, and other autoantibodies, including immunoglobulin, IgA, IgG and IgM, were negative; TB-AB and T-SPOT were negative. Enhanced abdominal CT showed thickening of the ileocecal region, partial infiltration around the ascending colon, and a blurred surrounding fat space. Enteroscopy suggested a large ulcer in the ileocecal region, and the location of the ulcer was different from that on review in 2019. Giant ulcers showed a migratory appearance, as shown in Table 1 and Attachment 1(Figure1) . A satisfactory and accurate diagnosis could not be obtained by pathology at our hospital. Therefore, we seeked help from Wechat at Zhongnan Hospital of Wuhan University, which has a well-known inflammatory bowel disease research centre and a digital telepathology consultation centre.
The pathological results showed slight mucosal changes (crypt branching, twisting and elongation) from the ileocecal part to the descending colon, focal enhancement, inflammation, obvious crypt withering, ulcers in the ileocecal region, and prominent lymphocyte infiltration. Immunohistochemistry showed that the local CD3-positive T cells were dense, and the Ki-67-positive rate was 30%. In situ hybridization showed that EBV-encoded small nuclear RNA (EBER)+ cells reached 30/HPF as well as the following: CD2 (T cells+), CD20 (B cells+), CD30 (-), CD5 (T cells+), CD56 (scattered cells+), granzyme B (scattered cells+), and CD21 (FDC Net +). No pylorus gland metaplasia or granuloma was found. A pathological image is shown in Attachment 1(Figure2).
At this time, we performed an EBV serological examination, and the results showed the following: IgA negative (-), IgM negative (-), IgG positive (+), and peripheral blood EBV DNA concentration: 1.75* 10 ^ 3 copies/ml. Combined with the clinical manifestations, pathology and serological results, the final diagnosis was CAEAE.
The treatment regimen was as follows: prednisone (55 mg QD for four weeks, then reduced to one tablet (20 mg) a week and then to one tablet every two weeks until discontinuation). One week after treatment, the liver and kidney function tests and electrolyte levels were normal. The patient was followed up every 2 weeks, and no abnormalities were found. After 4 weeks of hormone therapy, the patient developed right abdominal pain with high fever and immediately went to the emergency department. Abdominal CT was performed to consider cavity/organ perforation. Laparotomy was performed immediately. Ileocecal perforation was found during the operation, and right hemicolectomy was performed. Postoperative pathology showed intestinal perforation (right colon). A large number of acute and chronic inflammatory cells infiltrated the intestinal wall with necrosis. Mesenteric lymph nodes showed reactive hyperplasia (18 pieces). EBER was positive in the surgical and pathological specimens. After the operation, the patient’s condition was unstable, with repeated high fever and occasional abdominal pain. Laboratory examinations showed a reduction in leukocytes, thrombocytopenia, and liver dysfunction. The patient died 32 days after the operation.