Author/Year Age/Sex Symptoms Signs/Laboratory examination Disease course Endoscopic findings Histopathologic findings Misdiagnosis PCR/VCA-IgM/VCA-IgG/EA/EBV DNA/EBER Serum EBV DNA (copies/ml) Medication/Surgery Follow-up and prognosis
Our case 49/M Abdominal pain CRP 38.67 mg/L, CA72-4 10.32 U/ml, UA 446 µmol/L 3 years Multiple ulcers in the colon Slight mucosal changes (crypt branching, twisting and elongation) from the ileocecal part to the descending colon, focal enhancement, inflammation, obvious crypt withering and ulcers in the ileocecal region, prominent lymphocyte infiltration. UC +/-/+/NT/NT/+/T 1.75× 10^3 Hormone plus gamma globulin/Emergency right hemicolectomy exploratory laparotomy Died 1 month after exploratory laparotomy
Yangxiao Zhou, 2020 50/M Abdominal pain, diarrhoea, high fever, Weight loss WBC 13.4 × 109/L, neutrophil ratio 0.8, Hb 120.0 g/L, PLT 289.0 × 109/L; ESR 31 mm/1H, CRP 90.2 mg/l, calcitonin To 0.46 μ g/L and ferritin > 2000 μ g/L 2 months Multiple ulcers of colon, Ileal aphthous ulcer acute and chronic inflammation with lymphoproliferation in the ileocecal mucosa, which was dominated by T lymphoproliferation and ulcerated IBD NT/NT/NT/NT/NT/+ 1.29×10^6 Intravenous methylprednisolone, glucocorticoids/NA 3 months, died of haemorrhagic shock, Gastrointestinal
51/M High fever, diarrhoea, abdominal pain,Haematochezia NA/procalcitonin 0.07 µg/L 1 months Multiple deep ulcers of colon and rectum Full thickness light moderate chronic inflammation of colonic mucosa IBD NT/NT/NT/NT/NT/+ 4.63×10^4 Intravenous methylprednisolone, glucocorticoids/NA Continuous follow-up
24/M Fever, rash, haematochezia Splenomegaly, Pelvic small lymph node shadow 3 years Multiple jejunal ulcer Moderate chronic inflammation of mucosa with lymphangiectasia IBD NT/NT/NT/NT/NT/+ 4.34×10^6 Intravenous methylprednisolone, glucocorticoids/NA loss to follow-up
Bo Zhang, 2020 39/M Abdominal pain, fever, adenopathy, diarrhoea, haematochezia Hb 12 g/L, inflammatory indicators increased, coagulation function altered Numerous irregular ulcers in the colon Lymphatic follicles in the lamina propria, crypt abscess in some glands, aggregation of atypical lymphoid cells UC NT/NT/NT/NT/NT/+ <5 × 10^3 - -
28/M Abdominal pain, fever, adenopathy, retrosternal pain, diarrhoea, splenomegaly Hb 92 g/L, inflammatory indicators increased, coagulation function altered Numerous shallow and small ulcers in the colon Tissue granulation, atypical lymphocyte infiltration IBD NT/NT/NT/NT/NT/+ 3.8 × 10^4 - -
48/F Abdominal pain, fever, adenopathy Hb 105 g/L, inflammatory indicators increased, coagulation function altered Numerous irregular ulcers in the colon Granulomatous tissue and lymphoid tissue hyperplasia, atypical lymphocyte infiltration CD NT/NT/NT/NT/NT/+ NA - -
Dong Xuyang, 2018 62/M Abdominal pain, haematochezia, high fever, diarrhoea Haemoglobin decreased, RBC decreased, WBC decreased, thrombocytopenia, coagulation function altered 2 months Multiple deep ulcers in the colon Acute and chronic inflammation IBD -/-/+/+/-/+ 3.8× 10^3 Ganciclovir, glucocorticoid, imipenem, cilastatin, vancomycin/NA 2 weeks, septic shock, death
27/M High fever, diarrhoea, haematochezia, abdominal pain Haemoglobin decreased, RBC decreased, WBC decreased, thrombocytopenia, coagulation function altered 2 months Multiple deep ulcers in the colon Multiple ulcerations of the colon with lymphocytic and plasmacytic infiltration CD -/-/+/+/+/+ 9× 10^4 Glucocorticoid, intravenous immunoglobulin/emergency right hemicolectomy 2 weeks, haemorrhagic shock, death
28/M High fever, haematochezia, abdominal pain, diarrhoea Haemoglobin decreased, RBC decreased, WBC decreased, thrombocytopenia, coagulation function altered 5 months Multiple ulcers in the colon and ileum Acute and chronic inflammation CD -/-/+/+/+/+ 5× 10^5 Ganciclovir, glucocorticoids/NA 6 months, perforation, death
32/F Abdominal pain, diarrhoea, high fever, haematochezia Haemoglobin decreased, RBC decreased,coagulation function altered 14 years Multiple ulcers in the colon, duodenum and ileum Acute and chronic inflammation, focal lymphocyte accumulation, focal crypt architectural irregularity UC -/-/+/+/-/+ 8.9× 10^4 Glucocorticoid/NA 2.5 months, death
29/M Abdominal pain, dyssynergistic defecation, high fever, haematochezia Haemoglobin decreased, RBC decreased,coagulation function altered 4 years Multiple small intestinal ulcers Severe stenosis of the small intestine with perforation, mucosal ulceration with bleeding and fibrosis, necrosis and granulation tissue seen on the serosal surface, abscess formation in the mesentery, and chronic inflammation of lymph nodes CD -/-/+/±/-/+ 2.5× 10^5 Intravenous immunoglobulin, methotrexate, CHOEP regimen for 3 months/partial resection of the small intestine 13 months, died of haemorrhagic shock
26/F High fever, diarrhoea, abdominal pain, haematochezia Haemoglobin decreased, RBC decreased,coagulation function altered 2 years Multiple ulcers in the small intestine, duodenum and ileum Extensive ulceration, disorganized crypt architecture, chronic inflammatory cell infiltration of the intestinal wall, chronic inflammation of periintestinal lymph nodes IBD -/+/+/-/-/+ 4.5× 10^4 Glucocorticoids, cefoperazone sodium sulbactam sodium, vancomycin/duodenectomy and partial jejunectomy 2 weeks, died of haemorrhagic shock
Rongbei Liu, 2018 72/M Watery stool, intermittent fever (>39 °C) Hepatomegaly, splenomegaly, lymphadenopathy/increased WBC, PLT, CRP and ESR, OB(+) - Numerous shallow and small ulcers in the colon Dense lymphocytic infiltrate in the lamina propria, transmural inflammation, aggregation of lymphocytes NT/-/+/NT/NT/+ 2.30 × 10^4 Steroids and antibiotics/YES Died
21/M Watery stool, intermittent fever (>39 °C) Hepatomegaly, splenomegaly/increased WBC, PLT, CRP and ESR, OB(+) - Numerous irregular ulcers in the colon Dense lymphocytic infiltrate in the lamina propria, intraepithelial lymphocytosis, fissuring ulcers, crypt abnormalities NT/-/+/NT/NT/+ 8.84 × 10^6 Steroids, mesalazine, and antibiotics/YES Died
50/F Watery stool, intermittent fever (>39 °C) Hepatomegaly, splenomegaly/increased WBC, PLT, CRP and ESR, OB(+) - Numerous ulcers in the small intestine Intraepithelial lymphocytosis, crypt abnormalities, intraepithelial lymphocytosis NT/-/+/NT/NT/+ 2.55 × 10^6 Mesalazine and antibiotics/YES Died
50/M Bloody stool, intermittent fever (>39 °C) Splenomegaly/increased WBC, PLT, CRP and ESR, OB(+) - Numerous small ulcers in the colon and small intestine Dense lymphocytic infiltrate in the lamina, transmural inflammation, fissuring ulcers NT/-/+/NT/NT/+ 3.01 × 10^4 Mesalazine and steroids/YES Survived
70/F Intermittent fever Splenomegaly/increased WBC, PLT, CRP and ESR, OB(+) - NA Dense lymphocytic infiltrate in the lamina, crypt abnormalities, transmural inflammation, fissuring ulcers NT/-/+/NT/NT/+ NA Mesalazine and antibiotics/YES Survived
40/M Bloody stool, intermittent fever (>39 °C) Splenomegaly, lymphadenopathy/increased WBC, PLT, CRP and ESR, OB(+) - Numerous irregular ulcers in the colon Crypt abnormalities NT/-/+/NT/NT/+ 4.40 × 10^4 Mesalazine, steroids, and antibiotics/NO Survived
57/M Intermittent fever (>39 °C) Splenomegaly, lymphadenopathy/increased WBC, PLT, CRP and ESR, OB(+) - Numerous irregular ulcers in the colon Dense lymphocytic infiltrate in the lamina, intraepithelial lymphocytosis, crypt abnormalities NT/-/+/NT/NT/+ 8.17 × 10^3 Mesalazine and steroids/NO Survived
30/M Bloody stool, Intermittent fever (>39 °C) Lymphadenopathy/increased WBC, PLT, CRP and ESR, OB(+) - Numerous irregular ulcers in the colon Intraepithelial lymphocytosis NT/-/+/NT/NT/+ 7.06 × 10^6 Mesalazine, steroids, and antibiotics/NO Survived
12/M Intermittent fever (>39 °C Lymphadenopathy/increased WBC, PLT, CRP and ESR, OB(+) - Numerous irregular ulcers in the colon, a huge irregular ulcer in the ileocecal junction Dense lymphocytic infiltrate in the lamina NT/-/+/NT/NT/+ 4.11 × 10^4 Mesalazine, steroids, and antibiotics/NO Survived
12/M Watery stool, Intermittent fever (>39 °C) Lymphadenopathy/increased WBC, PLT, CRP and ESR, OB(+) - Huge irregular ulcer in the ileocecal junction Dense lymphocytic infiltrate in the lamina NT/-/+/NT/NT/+ NA Mesalazine, steroids, antibiotics, and traditional Chinese medicine/NO Survived
34/F Bloody stool, intermittent fever (>39 °C) Lymphadenopathy/increased WBC, PLT, CRP and ESR, OB(+) - Numerous ulcers in the colon Intraepithelial lymphocytosis NT/-/+/NT/NT/+ 5.71 × 10^5 Mesalazine, steroids, and antibiotics/NO Survived
Yu Zhang, 2017 55/M Abdominal pain, diarrhoea, high fever, bloody stool Mild splenomegaly/increased WBC, CRP, OB(+) 3 years Multiple superficial polymorphic ulcers covered with pus Moss all over the terminal ileum and colon, resulted in partial luminal stenosis Chronic active inflammation with a large amount of neutrophils and lymphocytes infiltrated with a normal tissue structure, partial cryptitis IBD +/NT/NT/NT/NT/+ NA _ _
Si Wei, 2016 55/M Recurrent fever with diarrhoea Increased WBC 11 years Diffuse patchy ulceration of the mucosa of the transverse, descending and sigmoid colons Oedema of the colorectal mucosa, lymphocyte and plasma cell infiltration, crypt architectural changes were not evident, cryptitis and crypt abscesses occasionally seen, ulcer formation IBD NA/+ NA _ _
Antonio Cuadrado Lav í n, 2008 49/M Worsening epigastric pain, Decreased haemoglobin 16 days Multiple ulcerated lesions and white-yellow pseudomembranes in the second duodenal portion Ulcerated lesions with ulcerated lesions with a lymphocytic and polymorphonuclear inflammatory infiltrate +/-/+/NT/+/+ NA