Journal Article

Chronic active Epstein-Barr virus infection of T-cell type, systemic form in an African migrant: case report and review of the literature on diagnostics standards and therapeutic options

Wass, Maxi, Bauer, Marcus, Odparlik, Andreas, Lorenz, Kerstin, Pfannes, Roald, Müller, Lutz P, Wickenhauser, Claudia

2018

Overview

Background Chronic active Epstein-Barr virus (EBV) infection (CAEBV) of the T-/NK-cell type, systemic form is a rare and potentially life-threatening illness caused by persistent EBV infection. The highest incidence is found in children and adolescents with increased frequency among Asians and Native Americans, while the disease is uncommon in Western countries. Typically patients present with unspecific symptoms, like fever, lymphadenopathy, hepatosplenomegaly and liver dysfunction. Due to fatal complications including hemophagocytic syndrome, coagulopathy, multiple organ failure and development of EBV-positive lymphoproliferative disease (LPD) or lymphoma early diagnosis is critical for successful treatment. However, in consequence of the lack of experience due to the low incidence in Europe, a broad spectrum of clinical manifestations and a particularly unexpected group of patients, diagnosis can be challenging. Inhere we describe the clinicopathological findings of an African adult with CAEBV associated LPD with a brief review of the literature. Case presentation A 42-year-old African man with fever, enlargement of the spleen and a suspected epileptic seizure was referred to our hospital. Diagnostic testing repeatedly revealed a massive EBV-DNA load in peripheral blood. Whole-body PET-CT-scan presented a strong uptake at multiple bone marrow sites, the thyroid and the adrenal glands. Histopathological analysis of bone marrow and thyroid gland revealed a highly proliferating, atypical and predominantly intravascular cytotoxic T-cell population with intracellular EBV-encoded RNA. Clonality analysis revealed the presence of polyclonal T-cell-receptor. Based on these findings a CAEBV of the T-/NK-cell type, systemic form was diagnosed. Subsequent therapy including three cycles of chemotherapy with cyclophosphamide, doxorubicin, vincristine and prednisolone resulted in decreased EBV load, clinical improvement and ongoing complete remission. Conclusion Adult-onset CAEBV of T/NK-cell type usually comprises a poor prognosis and is extremely rare in Western countries. Therefore, our case highlights the need for a clinical awareness of this disease in patients with systemic illness and for a comprehensive multidisciplinary diagnostic approach to facilitate diagnosis. Treatment options include antiviral drugs, immunosuppressive agents and systemic chemotherapy with or without allogeneic stem cell transplantation. Given the limited data these options need to be decided upon in each patient individually considering severity of the disease, comorbidities and response.

• Publisher: BioMed Central,BioMed Central Ltd,Springer Nature B.V,BMC
• Subject: Adolescents / Adrenal glands / Adult / Adults / African Continental Ancestry Group / Antineoplastic Combined Chemotherapy Protocols - therapeutic use / Antiviral agents / Biomarkers / Biomedical and Life Sciences / Biomedicine / Biopsy / Bone marrow / Cancer Research / Care and treatment / Case Report / Case reports / Case studies / Chemotherapy / Children / Chronic active Epstein-Barr virus infection / Chronic Disease / Chronic infection / Convulsions & seizures / Cyclophosphamide / Cyclophosphamide - therapeutic use / Cytotoxicity / Deoxyribonucleic acid / Diagnosis / Diagnostic standards / DNA / Doxorubicin / Doxorubicin - therapeutic use / Epilepsy / Epstein-Barr virus / Epstein-Barr virus diseases / Epstein-Barr Virus Infections - diagnosis / Epstein-Barr Virus Infections - drug therapy / Epstein-Barr Virus Infections - immunology / Epstein-Barr Virus Infections - virology / Fever / Health Promotion and Disease Prevention / Hemophagocytic syndrome / Herpesvirus 4, Human - physiology / Hospitals / Humans / Illnesses / Immunophenotyping / Immunosuppressive agents / Infections / Liver diseases / Lymph Nodes - metabolism / Lymph Nodes - pathology / Lymphadenopathy / Lymphatic diseases / Lymphocytes / Lymphocytes T / Lymphoma / Male / Medical and radiation oncology / Medicine/Public Health / Multiple organ dysfunction syndrome / Oncology / Patients / Positron Emission Tomography Computed Tomography / Prednisone - therapeutic use / Ribonucleic acid / RNA / Spleen / Stem cell transplantation / Stem cells / Surgical Oncology / T-Lymphocyte Subsets - immunology / T-Lymphocyte Subsets - metabolism / T-Lymphocyte Subsets - virology / T/NK-cell lymphoproliferative disease / Thyroid / Thyroid gland / Tomography / Transients and Migrants / Transplants & implants / Treatment Outcome / Tropical diseases / Tumors / Vincristine - therapeutic use / Viral Load / Western countries