Viral infections. Summary Summary. Symptoms Symptoms. These features may be different from person to person. Some people may have more symptoms than others and symptoms can range from mild to severe. This list does not include every symptom or feature that has been described in this condition. Over time, CAEBV can lead to failure of the immune system which, if not treated, can lead to potentially life-threatening complications. Showing of 8 View All. Permanent enlargement of the airways of the lungs.
Decreased immune function. Frequent respiratory infections. Multiple respiratory infections. Susceptibility to respiratory infections. Sinus inflammation. Increased spleen size. Do you have more information about symptoms of this disease?
We want to hear from you. Cause Cause. This allows the symptoms of an EBV infection to persist and get worse over time. Diagnosis Diagnosis. Treatment Treatment. Hematopoietic stem-cell transplant is currently the only curative treatment for this condition. Statistics Statistics. Do you have updated information on this disease? In some cases, the virus may reactivate. This does not always cause symptoms, but people with weakened immune systems are more likely to develop symptoms if EBV reactivates.
EBV spreads most commonly through bodily fluids, especially saliva. However, EBV can also spread through blood and semen during sexual contact, blood transfusions, and organ transplantations. EBV can be spread by using objects, such as a toothbrush or drinking glass, that an infected person recently used. The virus probably survives on an object at least as long as the object remains moist. The first time you get infected with EBV primary EBV infection you can spread the virus for weeks and even before you have symptoms.
Once the virus is in your body, it stays there in a latent inactive state. If the virus reactivates, you can potentially spread EBV to others no matter how much time has passed since the initial infection. Diagnosing EBV infection can be challenging because the symptoms are similar to other illnesses. In a case in which serial samples were obtained, clonal evolution of EBV-infected cells was confirmed with branching mutations in DDX3X. These results indicate that serial acquisition of mutations in EBV-infected NK or T cells have the potential to result in transformation of the cells and may contribute to lymphomagenesis in this disease.
Although no single genetic defect has been identified in CAEBV disease, a positive association with human leukocyte antigen HLA A26 and a negative association with B52 were observed Interestingly, both the A26 and B52 alleles are frequently seen in East Asia and Mexico, where the prevalence of the disease is high. Associations with HLA loci have been reported in other EBV-associated malignancies that show geographically distinct distributions 49 , Lymphadenopathy and splenomegaly were the most frequent signs and symptoms, followed by fever, hepatitis, hypogammaglobulinemia, pancytopenia, hemophagocytosis, and hepatomegaly.
Less common symptoms included pneumonitis, central nervous system disease, and periphery neuropathy. Some patients had B cell lymphopenia, others had reduced numbers of NK cells, and some had low numbers of both cells.
Deaths were most often due to progressive EBV lymphoproliferative disease or opportunistic infections. EBV-infected T or NK cells usually express cytotoxic molecules, such as perforin, granzyme, and T-cell intracytoplasmic antigen TIA -1 51 , 52 , indicating that they have a cytotoxic cell phenotype.
Typically in Asia, patients develop fever, hepatosplenomegaly, and lymphadenopathy; other common symptoms are thrombocytopenia, anemia, skin rash, diarrhea, and uveitis Interstitial pneumonia, calcifications in basal ganglia, and coronary aneurysms are occasionally seen without any symptoms. Some patients may have skin symptoms, such as hypersensitivity to mosquito bites and hydroa vacciniforme.
In the absence of treatment, patients with CAEBV develop progressive cellular and humoral immunodeficiencies and develop opportunistic infections, hemophagocytosis, multi-organ failure, or EBV-positive B, T, or NK cell lymphomas CAEBV is refractory to antiviral therapy, interferon, intravenous immunoglobulin, and conventional chemotherapy and thus has a poor prognosis.
Many other treatments have been tried including immunosuppressive agents such as cyclosporine or corticosteroids, autologous EBV-specific cytotoxic T cells, rituximab in the case of B cell CAEV, and the combination of bortezomib and ganciclovir.
In some cases, these other treatments have resulted in transient reductions in systemic symptoms with improvement in laboratory abnormalities; however, the disease eventually returns and patients succumb to their disease if they do not undergo hematopoietic stem cell transplantation.
Hematopoietic stem cell transplantation alone is a curative treatment for the disease, although the incidence of transplantation-related complications is high 54 , All authors listed have made a substantial, direct, and intellectual contribution to the work and approved it for publication.
The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. We thank Dr. National Center for Biotechnology Information , U. Front Immunol. Published online Dec Hiroshi Kimura 1 and Jeffrey I. Jeffrey I. Author information Article notes Copyright and License information Disclaimer. Edited by: Stuart G.
Cohen, vog. Specialty section: This article was submitted to Primary Immunodeficiencies, a section of the journal Frontiers in Immunology. Received Sep 9; Accepted Dec 8.
Copyright At least a portion of this work is authored by Jeffrey I. Cohen on behalf of the U. Government and, as regards Dr. Cohen and the US government, is not subject to copyright protection in the United States. Foreign and other copyrights may apply. The use, distribution or reproduction in other forums is permitted, provided the original author s or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice.
No use, distribution or reproduction is permitted which does not comply with these terms. This article has been cited by other articles in PMC. Introduction Primary infection of adolescents and young adults often results in infectious mononucleosis with fever, lymphadenopathy, and sore throat 1. N Engl J Med. Acute progressive Epstein-Barr virus infections. Ann Rev Med. Clinical and virologic characteristics of chronic active Epstein-Barr virus infection.
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