Four Cases Report Of EBV-Associated Lyphoproliferative Disorder And A Review Of The Literature

ObjectionIt's difficult to make diagnosis and treatment of EBV-associated lymphoproliferative disorder,which clinical manifestations and state of illness are different. This article aims to analyze the clinical characteristics of the disease, clinical manifestations, diagnosis and treatment methods to improve the overall understanding of the disease for doctors and it's help for early diagnosis and treatment.MethodsThis study retrospective analyzed the clinical data of four patients with EBV-associated lymphoproliferative disorder in dermatological department of Qilu hospital from 2010 to 2015 to analysis pathogenesis, clinical manifestations, examination methods, treatment and follow-up of EBV+-LPD.Retrieval reported cases of that published in the database of CNKI,wanfang,Pubmed at home and abroad from 2010 to 2015,at the same time, analysis 52 cases of EBV+T/NK-LPD,which recorded more detailed information at home.ResultsFour cases of the patient's condition in our hospital1.General information:In 4 patients,3 males and 1 female. Age range from 5 years to 13 years and 9 months.2.1nducement:Two cases no obvious incentive, one case is exposed to the sun and one case with mosquito bites as an incentive.3. Clinical manifestations:Common clinical manifestations are skin rash, fever, iritis, enlarged lymph nodes, abdominal pain, enlarged tonsil.4. Laboratory tests:Four cases were detected positive for EBV, other common abnormalities included routine blood, liver function, humoral immune, elevated IgE levels and so on.5. Treatment and prognosis:In four cases, one case with symptomatic treatment, but the rash is still repeated, one cases treated combine prednisone therapy and antiviral therapy, At present, the patient's skin lesions have healed and no new lesions.Two cases with a small dose of the prednisone in combination with interferon therapy, Both are in stable condition and no new lesions currently.Up to now, four patients still under follow-up.The condition of the cases reported in China1. General information:In 52 cases,33 male patients and 19 female patients, male to female ratio is 1.74:1. The youngest is 1 year old, the oldest is 53 years old. The average age of patients was 17.29 years. The median age of onset was 10 years. The shortest duration is 15 days, the longest duration is 45 years, average duration is 3.48 years. There are 21 cases of adults and 31 cases of children, children and adult prevalence ratio is 1.48:1. The peak incidence of EBV+TNK-LPD in the range of 7 to 14 years old.2.Inducement:8 cases is exposed to the sun as an incentive,4 case with mosquito bites as an incentive, Two cases is cold as an incentive, One case is fatigue as an incentive,One case with seafood consumption as incentives, One case in contact with contaminated water as an incentive, the other cases no obvious incentives.3. Clinical manifestations:The most common clinical manifestations were Fever in 49 patients (94.23%%),rash in 31 cases (59.62%), Lymphadenopathy and splenomegaly in 27 cases(51.92%) respectively, hepatomegaly in 22 cases (42.30%).4. Laboratory tests:EBV detection (including EBV-DNA detection, EBV-specific antibody detection and tissue EBER detection) were positive, followed by abnormal liver function, abnormal peripheral blood.5. Treatment and Prognosis:Mainly in combination therapy. To select two or more joint treatment from treatment with corticosteroids, anti-virus, interferon, immune globulin, treatment effect is best. In 52 cases,16 cases died, the mortality rate of 30.77%.Common causes of death were hemophagocytic syndrome, malignant lymphoma, infections and so on.Conclutions1.EBV+T/NK-LPD is found mostly in Asia.Age of onset of EBV+T/NK-LPD were young, more men than women.2.The onset of EBV+T/NK-LPD may be associated with EBV infection, patients' immune abnormalities, environmental factors, mosquito bites and so on.3.EBV+-LPD have many kinds of clinical manifestations and often involving multi-organ, multi-system.4.Combined with clinical manifestations of EBV related tests(EBER, peripheral blood EBV-DNA quantitative, EBV antibody, EBV terminal repeat sequence analysis) and histopathological and immunohistochemical examination is helpful to make a early diagnosis.5.The disease mortality rate is high, the prognosis is poor. There is no specific treatmentTo select treatment with glucocorticoid, interferon, antivirus, immune globulin combination can slow disease progression. Targeted therapy become a new ideas of Epstein-Barr virus related diseases.