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An Efficient and Simple Method to Establish NK and T Cell Lines from Patients with Chronic Active Epstein-Barr Virus Infection
Published on: March 30, 2018
Chronic active EBV infection with features of granulomatosis with polyangiitis
Masaki Shimomura1, Hideaki Morishita1, Takaaki Meguro1
1Department of Allergy and Clinical Immunology, Shizuoka Children's Hospital, Shizuoka, Japan.
Insights
This case highlights chronic active Epstein-Barr virus (CAEBV) infection mimicking granulomatosis with polyangiitis (GPA) in a pediatric patient. Accurate diagnosis is crucial due to overlapping clinical and pathological features.
Area of Science:
- Pediatric Rheumatology
- Infectious Diseases
- Immunology
Background:
- Granulomatosis with polyangiitis (GPA) is a rare autoimmune vasculitis.
- Differential diagnosis is essential in complex pediatric cases presenting with systemic inflammation.
Observation:
- A 13-year-old boy presented with recurrent leg ulcers, sinusitis, and weight loss.
- Initial biopsy suggested small vessel vasculitis and granulomatous inflammation, consistent with GPA.
Findings:
- Further analysis revealed Epstein-Barr virus-encoded RNA (EBER) in lymphocytes, predominantly CD4+ T cells.
- Elevated EBV-DNA load confirmed chronic active EBV infection (CAEBV).
Implications:
- This case underscores the importance of considering CAEBV in pediatric patients with suspected vasculitis.
- Distinguishing CAEBV from GPA is critical for appropriate treatment and management.
Abstract:
Herein, we report the case of a 13-year-old boy with multiple recurrent ulcers on his legs. He developed severe sinusitis at 10 years of age and had significant weight loss (6 kg) in the 2 months prior to admission. Histology of tissue biopsied from the ulcer indicated small vessel vasculitis and granulomatous inflammation. Given that these findings met the diagnostic criteria for granulomatosis with polyangiitis (GPA), he was treated with immunosuppressive agents. Further pathology, however, indicated Epstein-Barr virus (EBV)-encoded RNA (EBER) in most lymphocytes in the same sample. The EBER-positive lymphocytes were mainly CD4-positive T cells. The EBV-DNA load in the peripheral blood was also abnormally increased (1.0 × 10(4) copies/μg DNA). Thus, the diagnosis was established as chronic active EBV infection (CAEBV). This case illustrates the necessity of careful differential diagnosis of CAEBV owing to its clinical resemblance and pathological overlap with GPA.
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