A 20-month-old male infant with multiorgan dysfunction after Epstein-Barr virus(EBV)infection developed Reye’s syndrome.He also suffered from acute liver failure,life-threatening cerebral edema,severe disseminated in...A 20-month-old male infant with multiorgan dysfunction after Epstein-Barr virus(EBV)infection developed Reye’s syndrome.He also suffered from acute liver failure,life-threatening cerebral edema,severe disseminated intravascular coagulation(DIC),and myocardial involvement.EBV infection aggravated the progress of Reye’s syndrome,leading to death despite full supportive and symptomatic therapy.This critical case suggested that pediatricians should pay attention to multiorgan involvement of severe EBV infection.展开更多
Globally,a shift in the epidemiology of chronic liver disease has been observed.This has been mainly driven by a marked decline in the prevalence of chronic hepatitis B virus infection(CHB),with the greatest burden re...Globally,a shift in the epidemiology of chronic liver disease has been observed.This has been mainly driven by a marked decline in the prevalence of chronic hepatitis B virus infection(CHB),with the greatest burden restricted to the Western Pacific and sub-Saharan African regions.Amidst this is a growing burden of metabolic syndrome(MetS)worldwide.A disproportionate co-burden of human immunodeficiency virus(HIV)infection is also reported in sub-Saharan Africa,which poses a further risk of liver-related morbidity and mortality in the region.We reviewed the existing evidence base to improve current understanding of the effect of underlying MetS on the development and progression of chronic liver disease during CHB and HIV co-infection.While the mechanistic association between CHB and MetS remains poorly resolved,the evidence suggests that MetS may have an additive effect on the liver damage caused by CHB.Among HIV infected individuals,MetS-associated liver disease is emerging as an important cause of non-AIDS related morbidity and mortality despite antiretroviral therapy(ART).It is plausible that underlying MetS may lead to adverse outcomes among those with concomitant CHB and HIV co-infection.However,this remains to be explored through rigorous longitudinal studies,especially in sub-Saharan Africa.Ultimately,there is a need for a comprehensive package of care that integrates ART programs with routine screening for MetS and promotion of lifestyle modification to ensure an improved quality of life among CHB and HIV coinfected individuals.展开更多
Background: Adult hemophagocytic syndrome is a critical condition that is often difficult to diagnose and results in a bad prognosis because of the lack of effective and unified treatment. Aim: To investigate the clin...Background: Adult hemophagocytic syndrome is a critical condition that is often difficult to diagnose and results in a bad prognosis because of the lack of effective and unified treatment. Aim: To investigate the clinical, diagnosis and treatment of hemophagocytic syndrome. Case Presentation: A 22-year-old female patient received a splenectomy, and the spleen was sent to the King Med Center of Medical Inspection. The results confirmed the diagnosis of hemophagocytic syndrome related to EB (Epstein-Barr) virus infection. Conclusion: Early diagnosis and early intervention are important factors for affecting the progression of the disease and improving its prognosis.展开更多
The Epstein-Barr virus(EBV)has an important and multifaceted role in liver pathology.As a member of the herpes virus family,EBV establishes a persistent infection in more than 90%of adults.Besides acute hepatitis duri...The Epstein-Barr virus(EBV)has an important and multifaceted role in liver pathology.As a member of the herpes virus family,EBV establishes a persistent infection in more than 90%of adults.Besides acute hepatitis during primary infection,many clinical syndromes of interest for the hepatologist are associated with EBV infection.The role of EBV in the evolution of chronic hepatitis from hepatotropic viruses is considered.Chronic EBVassociated hepatitis is suspected in immunocompetent adults with compatible serology,suggestive histology and detection of the viral genome in the liver and/or increase of specific circulating cytotoxic T-lymphocytes.EBV is the main cause of post-transplant lymphoproliferative disorders which occur in up to 30%of cases.EBV-driven lymphoproliferative diseases are also recognized in non-immunocompromised patients and liver is involved in up to a third of the cases.Directly implicated in the pathogenesis of different tumors,EBV has a disputable role in hepatocellular carcinoma carcinogenesis.Further research is required in order to establish or reject the role of EBV in human liver cancer.This paper attempts to discuss the range of EBV-associated chronic liver diseases in immunocompetent patients,from mild,self-limiting mononuclear hepatitis to liver cancer.展开更多
BACKGROUND Chronic active Epstein-Barr virus infection(EBV)is a systemic EBV-positive lymphoproliferative disease,which may lead to fatal illness.There is currently no standard treatment regimen for chronic active EBV...BACKGROUND Chronic active Epstein-Barr virus infection(EBV)is a systemic EBV-positive lymphoproliferative disease,which may lead to fatal illness.There is currently no standard treatment regimen for chronic active EBV(CAEBV),and hematopoietic stem cell transplantation is the only effective treatment.We here report a CAEBV patient treated with PEG-aspargase,who achieved negative EBV-DNA.CASE SUMMARY A 33-year-old female Chinese patient who had fever for approximately 3 mo was admitted to our hospital in December 2017.EBV-DNA was positive with a high copy number.She was diagnosed with chronic active EB virus infection.PEGaspargase was administered at a dose of 1500 U/m2 at a 14-d interval,resulting in eradication of EBV for more than 6 mo.The effect of PEG-aspargase in this patient was excellent.CONCLUSION A chemotherapy regimen containing PEG-aspargase for CAEBV may be further considered.展开更多
BACKGROUND Because of atypical clinical symptoms,lymphoma is easily confused with infectious diseases.Extranodal nasal-type natural killer/T-cell lymphoma(NKTL)is more common,and there are few cases of eyelid site ons...BACKGROUND Because of atypical clinical symptoms,lymphoma is easily confused with infectious diseases.Extranodal nasal-type natural killer/T-cell lymphoma(NKTL)is more common,and there are few cases of eyelid site onset and intracranial infiltration,which increases the difficulty of diagnosis.This disease usually has a very poor prognosis and there are few reports of recovery.CASE SUMMARY A 3-year-old boy was admitted to our hospital due to an initial misdiagnosis of"eyelid cellulitis"and failed antibiotic treatment.He was characterized by fever,right eyeball bulging,convulsions,and abnormal liver function.His blood Epstein-Barr virus(EBV)DNA was positive(8.798×10^4 copies/mL),and remained positive for about half a year.The cranial imaging examination suggested a space-occupying lesion in the right eyelid,with the right temporal lobe and meninges involved.The boy underwent ocular mass resection.The pathological diagnosis was NKTL.He was diagnosed as having NKTL with intracranial infiltration,combined with chronic active EBV infection(CAEBV).Then he underwent systemic chemotherapy and intrathecal injection.The boy suffered from abnormal blood coagulation,oral mucositis,diarrhea,liver damage,and severe bone marrow suppression but survived.Finally,the tumor was completely relieved and his blood EBV-DNA level turned negative.The current follow-up has been more than 2 years and his condition is stable.CONCLUSION This case suggests that chemotherapy combined with intrathecal injection may have a good effect on intracranial infiltrating lymphoma and CAEBV,which deserves further study and discussion.展开更多
We report a case of natural killer(NK)/T-cell lymphoma with concomitant syndrome of inappropriate antidiuretic hormone secretion(SIADH).The patient was a 64-yearold woman with a history of nasopharyngeal carcinoma of ...We report a case of natural killer(NK)/T-cell lymphoma with concomitant syndrome of inappropriate antidiuretic hormone secretion(SIADH).The patient was a 64-yearold woman with a history of nasopharyngeal carcinoma of over 30 years.She was admitted with a chief complaint of intermittent fever for 2 mo.Palpation after admission indicated a swollen lymph node below the left jaw.Multiple imaging examinations on admission indicated multiple enlarged lymph nodes throughout the body.We performed a left submandibular lymph node biopsy,and the results revealed NK/T-cell lymphoma.A biochemical examination indicated Epstein-Barr virus positivity.At the same time,the patient developed hyponatremia.Based on her laboratory examination and clinical manifestation,decreased plasma osmolality,urine osmolality greater than plasma osmolality,lack of skin swelling,normal blood pressure,normal renal function,no adrenal function detected on serology,and no abnormalities in imaging examination of the adrenal glands,the likelihood of SIADH in the patient was high.After fluid restriction and administration of sodium chloride,the patient’s blood sodium level gradually increased.Subsequently,the immune function of the patient declined,there were severe symptoms of infection,and she died of respiratory failure.NK/T-cell lymphoma associated with SIADH has not,to our knowledge,been previously reported in PubMed.This case emphasizes the importance of monitoring serum ion levels,especially serum sodium,in patients with NK/T-cell lymphoma.展开更多
A 28-year-old woman with untreated autoimmune disorder, demonstrated skin rash and fever after taking Amoxicillin-clavulanate and developed progressive jaundice. A bone marrow aspiration indicated an increased number ...A 28-year-old woman with untreated autoimmune disorder, demonstrated skin rash and fever after taking Amoxicillin-clavulanate and developed progressive jaundice. A bone marrow aspiration indicated an increased number of macrophages with hemophagocytosis and liver biopsy showed pure centrilobular cholestasis with necrosis and some absence of portal bile ducts. Furthermore, a serological test for Epstein-Barr virus was positive. Under treatment by liver dialysis and administration of steroids led to rapidly defervescence and clinical improvement. However, liver enzymes were still markedly elevated with persistent anemia, even after immunosuppressive treatment. The patient is currently waiting for liver transplantation. This is the ?rst description of vanishing bile duct syndrome combined with hemophagocytic lymphohistiocytosis, with underlying causes including infection, drug-induced factors and untreated autoimmune disorder.展开更多
Hemophagocytic lymphohistiocytosis(HLH)is a cytokine storm syndrome caused by an overactive but ineffective immune reaction.Without prompt diagnosis and treatment,HLH is life-threatening.However,presenting symptoms ar...Hemophagocytic lymphohistiocytosis(HLH)is a cytokine storm syndrome caused by an overactive but ineffective immune reaction.Without prompt diagnosis and treatment,HLH is life-threatening.However,presenting symptoms are often nonspecific,with fatigue and fever being the most common.A high index of suspicion is therefore critical for early diagnosis and timely management.A previously healthy,65-year-old female who initially presented with fever and abdominal pain developed abdominal compartment syndrome(ACS)requiring decompressive laparotomy on hospital day 6.Intraoperative frozen sections of biopsied liver showed intense portal lymphohistiocytic infiltrates.Epstein-Barr virus DNA copy numbers escalated from 600 copies/mL after admission to 134,000 copies/mL before death.The diagnostic criteria of HLH-2004 were met.Patient expired on hospital day 12.It is important to raise awareness of ACS being an unusual presentation of HLH.Recent changes in diagnostic criteria tailored to adult HLH cases are reviewed.展开更多
目的:从氨基酸代谢角度探讨乙型肝炎病毒相关慢加急性肝衰竭(HBV-ACLF)湿热瘀黄证和气虚瘀黄证的生物学基础。方法:采用靶向氨基酸代谢组学分析HBV-ACLF湿热瘀黄证组(20例)与气虚瘀黄证组(20例)患者的血清氨基酸代谢谱,寻找两种证型的...目的:从氨基酸代谢角度探讨乙型肝炎病毒相关慢加急性肝衰竭(HBV-ACLF)湿热瘀黄证和气虚瘀黄证的生物学基础。方法:采用靶向氨基酸代谢组学分析HBV-ACLF湿热瘀黄证组(20例)与气虚瘀黄证组(20例)患者的血清氨基酸代谢谱,寻找两种证型的氨基酸代谢差异特征,分析不同证型中氨基酸代谢与炎症细胞因子表达的相关性。结果:湿热瘀黄证患者体内异亮氨酸表达水平显著高于气虚瘀黄证[(10.36±4.47)μg/ml vs(7.76±4.08)μg/ml,P=0.016],但色氨酸则表达相反[(10.26±4.95)μg/ml vs(14.51±7.32)μg/ml,P=0.014];湿热瘀黄证患者白细胞介素(IL)-6显著高于气虚瘀黄证[10.15(3.88,24.42)pg/ml vs 7.73(3.93,18.83)pg/ml,P=0.017],而IL-2、IL-10显著低于气虚瘀黄证[4.40(2.88,8.55)pg/ml vs 6.60(2.80,15.50)pg/ml,P=0.033;7.19(4.52,15.04)pg/ml vs 8.21(2.19,14.64)pg/ml,P=0.015];多种氨基酸代谢与两种证型的炎症细胞因子表达密切相关,尤其是色氨酸代谢与支链氨基酸代谢;湿热瘀黄证中氨基酸代谢主要与促炎细胞因子IL-2、IL-6相关,按照相关系数大小依次为天冬氨酸、谷氨酸、异亮氨酸、亮氨酸、蛋氨酸、酪氨酸、丝氨酸、赖氨酸、谷氨酰胺、色氨酸、苯丙氨酸;气虚瘀黄证中氨基酸代谢与肿瘤坏死因子α(TNF-α)、IL-2、IL-6、IL-10相关,按照相关系数大小依次为天冬氨酸、甘氨酸、丙氨酸、苏氨酸、色氨酸、丝氨酸。异亮氨酸及色氨酸代谢不仅在两种证型间差异有统计学意义,且差异性关联炎症细胞因子的表达。结论:HBV-ACLF湿热瘀黄证和气虚瘀黄证患者在氨基酸代谢及炎症反应中均具有异质性,且两者之间相互关联。色氨酸以及支链氨基酸代谢可能对HBV-ACLF的免疫炎症反应具有重要调节作用,与湿热瘀黄证和气虚瘀黄证的生物学基础密切相关。展开更多
Background:Severe fever with thrombocytopenia syndrome(SFTS)is an emerging hemorrhagic fever caused by a tick-borne bunyavirus SFTSV with case fatality up to 30%.The reactivation of Epstein-Barr virus(EBV)has been pro...Background:Severe fever with thrombocytopenia syndrome(SFTS)is an emerging hemorrhagic fever caused by a tick-borne bunyavirus SFTSV with case fatality up to 30%.The reactivation of Epstein-Barr virus(EBV)has been proven to occur in individuals with various immune suppression conditions.Methods:Here,we diagnosed 22 SFTSV infected patients with PCR in a hospital in Shandong Province,China in 2020.To understand the consequences of SFTSV infection leading to EBV reactivation,we examined EBV reactivation in SFTSV-infected patients with PCR and RT-PCR.Results:We found that EBV was reactivated in 18.2%(4/22)of SFTS patients,suggesting that EBV reactivation is common in SFTS patients.Compared with SFTS patients without EBV reactivation,SFTS patients with EBV-reactivation had a significantly lower median level of serum albumin(32.45 g/L vs.26.95 g/L,p=0.03)and a significantly higher median number of urine red blood cells(0 cells/μL vs.9 cells/μL,p=0.04).Conclusion:SFTS infection can reactivate EBV in patients,which may make the clinical condition of patients worsen.展开更多
Background Chronic active Epstein-Barr virus infection (CAEBV) has been previously reported to be sometimes associated with an aggressive clinical course. The characteristics of CAEBV in Mainland Chinese pediatric p...Background Chronic active Epstein-Barr virus infection (CAEBV) has been previously reported to be sometimes associated with an aggressive clinical course. The characteristics of CAEBV in Mainland Chinese pediatric patients are largely unreported. The main aims of this survey were to recognize the clinical features of CAEBV in children and to explore its diagnostic criteria and risk factors. Methods A retrospective study was performed on 53 pediatric patients (36 boys and 17 girls) with CAEBV who were admitted to Beijing Children's Hospital between 2003 and 2007. All their medical records were reviewed and analyzed. For each patient, demographic, clinical, laboratory data and outcome were collected. Independent-samples t test was used for statistical analysis. Results The age at onset of CAEBV was from 2 months to 14.6 years (mean (5.3_+3.3) years). At the time of onset, 43.4% patients had an infectious mononucleosis-like symptom. Most patients exhibited intermittent fever (92.5%, 49/53), hepatomegaly (81.1%, 43/53) and splenomegaly (77.4%, 41/53). Life-threatening complications including hemophagocytic syndrome (24.5%,13/53), interstitial pneumonia (24.5%, 13/53), hepatic failure (15.1%, 8/53) and malignant lymphoma (11.3%, 6/53) were also observed. The serum EBV DNA level in 23 patients with CAEBV was in the range of 5.05×10^2-4.60×10^6 copies/ml with a mean value of 103.7 copies/ml. Many patients with CAEBV generally had continuous symptoms during the observational period. Eleven out of 42 patients (26.2%) died 7 months to 3 years after onset. Deceased patients were more likely to have had lower platelet counts and albumin levels than the living patients (P 〈0.05 for all comparisons). Conclusions The study reveals that CAEBV in Chinese pediatric patients has a severe clinical course and prognosis is poor. Thrombocytopenia and decreases in albumin might potentially be risk factors for a poor prognosis. EBV loads should be measured and tissue should be stained on hybridization probes for EBV-encoded small RNA (EBER) if a patient presents with the known symptoms of CAEBV.展开更多
Background This study aimed to evaluate the feasibility and clinical effect of haploidentical hematopoietic stem cell transplantation(haplo-HSCT)for the treatment of pediatric patients with chronic active Epstein-Barr...Background This study aimed to evaluate the feasibility and clinical effect of haploidentical hematopoietic stem cell transplantation(haplo-HSCT)for the treatment of pediatric patients with chronic active Epstein-Barr virus infection(CAEBV).Methods Children with CAEBV who did not have matched donors and underwent haplo-HSCT in Beijing Children's Hospital,Capital Medical University,from October 2016 to June 2020 were analyzed retrospectively.Data relating to the clinical manifestations,engraftment,and prognosis of the children were extracted from medical records.Results Twenty-five patients,including 16 males and 9 females,with an onset age of 5.0±2.6 years and a transplantation age of 6.9±2.9 years,were enrolled irnhis study.The mean time from diagnosis to transplantation was 3.8(2.0-40.2)months.The mean observation time was 19.0±12.0 months.Three patients received the reduced intensity conditioning regimen,and the remaining patients all received the modified myeloablative conditioning regimen.By the end of the follow-up,23 patients were characterized by disease-free survival(DFS),22 were characterized by event-free survival(EFS).and two died.One of the patients died of thrombotic microangiopathy(TMA),and another died of graft versus host disease(GVHD);this patient discontinued the treatment for economic reasons.The 3-year overall survival(OS)rate was estimated to be 92.0%±5.4%,and the 3-year EFS rate was estimated to be 87.4%±6.8%.All active patients survived after HSCT event-free.Acute GVHD degrees 1-3 were observed in ten patients(40.0%),and degree IV was observed in six(24.0%),who were all cured except for one patient.Chronic GVHD was observed in nine(36.0%),and most of these cases were mild.The incidence of TMA and veno-occlusive disease(VOD)was 28.0%and 4.0%.Conclusions Haploidentical hematopoietic stem cell transplantation is safe and effective in the treatment of pediatric CAEBV and can be used as an alternative therapy without matched donors or emergency transplantation.Patients with active disease before HSCT also benefited from haplo-HSCT.Haplo-HSCT requires careful monitoring for complications,such as GVHD and TMA.Early detection of TMA and timely treatment can reduce mortality and can improve the survival rate.展开更多
Importance:Allogeneic hematopoietic stem cell transplantation(alloHSCT)is considered the only effective treatment for chronic active Epstein–Barr virus infection(CAEBV).The clinical efficacy and safety of allo-HSCT w...Importance:Allogeneic hematopoietic stem cell transplantation(alloHSCT)is considered the only effective treatment for chronic active Epstein–Barr virus infection(CAEBV).The clinical efficacy and safety of allo-HSCT with different conditioning regimens in children with CAEBV remain unclear.Objective:To evaluate the effectiveness and safety of allo-HSCT with the modified myeloablative conditioning(MAC)regimen for children with CAEBV and also the factors affecting the outcomes.Methods:We retrospectively analyzed children with CAEBV who underwent allo-HSCT with the modified MAC regimen at Beijing Children’s Hospital,Capital Medical University from October 2016 to June 2021.Data related to the clinical manifestations,engraftment,and outcome were extracted from the medical records.Results:The cohort comprised 41 patients(24 males,17 females)with a median transplantation age of 92.6(60.4,120.7)months and a median follow-up time of 28.2(15.3,40.2)months.Four patients(9.8%)died,among which three died from primary disease relapse,and one died from grade IV acute graft-versus-host diseases(aGVHD)after stopping treatment.The 3-year overall survival(OS)and 3-year event-free survival(EFS)rates were 88.8%±5.4%and 85.0%±5.7%,respectively.The 3-year OS and EFS did not significantly differ between the patients with hemophagocytic lymphohistiocytosis(HLH)and the patient without HLH(87.7%±6.8%vs.91.7%±8.0%,P=0.790;85.0%±6.9%vs.84.6%±10.0%,P=0.921),or among the patients with complete remission,partial remission,and activity disease before HSCT(all P>0.05).Multivariate analysis showed that grade III–IV aGVHD was a risk factor for mortality(Hazards ratio:11.65,95%confidence interval:1.00,136.06;P=0.050).Interpretation:Allo-HSCT with the modified MAC regimen is safe and effective for pediatric CAEBV.This treatment benefits patients with HLH or active disease.Patients with Grade III–IV aGVHD may be associated with worse outcomes.展开更多
文摘A 20-month-old male infant with multiorgan dysfunction after Epstein-Barr virus(EBV)infection developed Reye’s syndrome.He also suffered from acute liver failure,life-threatening cerebral edema,severe disseminated intravascular coagulation(DIC),and myocardial involvement.EBV infection aggravated the progress of Reye’s syndrome,leading to death despite full supportive and symptomatic therapy.This critical case suggested that pediatricians should pay attention to multiorgan involvement of severe EBV infection.
文摘Globally,a shift in the epidemiology of chronic liver disease has been observed.This has been mainly driven by a marked decline in the prevalence of chronic hepatitis B virus infection(CHB),with the greatest burden restricted to the Western Pacific and sub-Saharan African regions.Amidst this is a growing burden of metabolic syndrome(MetS)worldwide.A disproportionate co-burden of human immunodeficiency virus(HIV)infection is also reported in sub-Saharan Africa,which poses a further risk of liver-related morbidity and mortality in the region.We reviewed the existing evidence base to improve current understanding of the effect of underlying MetS on the development and progression of chronic liver disease during CHB and HIV co-infection.While the mechanistic association between CHB and MetS remains poorly resolved,the evidence suggests that MetS may have an additive effect on the liver damage caused by CHB.Among HIV infected individuals,MetS-associated liver disease is emerging as an important cause of non-AIDS related morbidity and mortality despite antiretroviral therapy(ART).It is plausible that underlying MetS may lead to adverse outcomes among those with concomitant CHB and HIV co-infection.However,this remains to be explored through rigorous longitudinal studies,especially in sub-Saharan Africa.Ultimately,there is a need for a comprehensive package of care that integrates ART programs with routine screening for MetS and promotion of lifestyle modification to ensure an improved quality of life among CHB and HIV coinfected individuals.
文摘Background: Adult hemophagocytic syndrome is a critical condition that is often difficult to diagnose and results in a bad prognosis because of the lack of effective and unified treatment. Aim: To investigate the clinical, diagnosis and treatment of hemophagocytic syndrome. Case Presentation: A 22-year-old female patient received a splenectomy, and the spleen was sent to the King Med Center of Medical Inspection. The results confirmed the diagnosis of hemophagocytic syndrome related to EB (Epstein-Barr) virus infection. Conclusion: Early diagnosis and early intervention are important factors for affecting the progression of the disease and improving its prognosis.
文摘The Epstein-Barr virus(EBV)has an important and multifaceted role in liver pathology.As a member of the herpes virus family,EBV establishes a persistent infection in more than 90%of adults.Besides acute hepatitis during primary infection,many clinical syndromes of interest for the hepatologist are associated with EBV infection.The role of EBV in the evolution of chronic hepatitis from hepatotropic viruses is considered.Chronic EBVassociated hepatitis is suspected in immunocompetent adults with compatible serology,suggestive histology and detection of the viral genome in the liver and/or increase of specific circulating cytotoxic T-lymphocytes.EBV is the main cause of post-transplant lymphoproliferative disorders which occur in up to 30%of cases.EBV-driven lymphoproliferative diseases are also recognized in non-immunocompromised patients and liver is involved in up to a third of the cases.Directly implicated in the pathogenesis of different tumors,EBV has a disputable role in hepatocellular carcinoma carcinogenesis.Further research is required in order to establish or reject the role of EBV in human liver cancer.This paper attempts to discuss the range of EBV-associated chronic liver diseases in immunocompetent patients,from mild,self-limiting mononuclear hepatitis to liver cancer.
基金Supported by National Natural Science Foundation of China,No.81871633Beijing Natural Science Foundation,No.7181003Beijing Municipal Administration of Hospitals’Ascent Plan,No.DFL20180101.
文摘BACKGROUND Chronic active Epstein-Barr virus infection(EBV)is a systemic EBV-positive lymphoproliferative disease,which may lead to fatal illness.There is currently no standard treatment regimen for chronic active EBV(CAEBV),and hematopoietic stem cell transplantation is the only effective treatment.We here report a CAEBV patient treated with PEG-aspargase,who achieved negative EBV-DNA.CASE SUMMARY A 33-year-old female Chinese patient who had fever for approximately 3 mo was admitted to our hospital in December 2017.EBV-DNA was positive with a high copy number.She was diagnosed with chronic active EB virus infection.PEGaspargase was administered at a dose of 1500 U/m2 at a 14-d interval,resulting in eradication of EBV for more than 6 mo.The effect of PEG-aspargase in this patient was excellent.CONCLUSION A chemotherapy regimen containing PEG-aspargase for CAEBV may be further considered.
基金Supported by the Beijing Hospital Authority"DengFeng"Talent Training Plan,No.DFL20180201.
文摘BACKGROUND Because of atypical clinical symptoms,lymphoma is easily confused with infectious diseases.Extranodal nasal-type natural killer/T-cell lymphoma(NKTL)is more common,and there are few cases of eyelid site onset and intracranial infiltration,which increases the difficulty of diagnosis.This disease usually has a very poor prognosis and there are few reports of recovery.CASE SUMMARY A 3-year-old boy was admitted to our hospital due to an initial misdiagnosis of"eyelid cellulitis"and failed antibiotic treatment.He was characterized by fever,right eyeball bulging,convulsions,and abnormal liver function.His blood Epstein-Barr virus(EBV)DNA was positive(8.798×10^4 copies/mL),and remained positive for about half a year.The cranial imaging examination suggested a space-occupying lesion in the right eyelid,with the right temporal lobe and meninges involved.The boy underwent ocular mass resection.The pathological diagnosis was NKTL.He was diagnosed as having NKTL with intracranial infiltration,combined with chronic active EBV infection(CAEBV).Then he underwent systemic chemotherapy and intrathecal injection.The boy suffered from abnormal blood coagulation,oral mucositis,diarrhea,liver damage,and severe bone marrow suppression but survived.Finally,the tumor was completely relieved and his blood EBV-DNA level turned negative.The current follow-up has been more than 2 years and his condition is stable.CONCLUSION This case suggests that chemotherapy combined with intrathecal injection may have a good effect on intracranial infiltrating lymphoma and CAEBV,which deserves further study and discussion.
文摘We report a case of natural killer(NK)/T-cell lymphoma with concomitant syndrome of inappropriate antidiuretic hormone secretion(SIADH).The patient was a 64-yearold woman with a history of nasopharyngeal carcinoma of over 30 years.She was admitted with a chief complaint of intermittent fever for 2 mo.Palpation after admission indicated a swollen lymph node below the left jaw.Multiple imaging examinations on admission indicated multiple enlarged lymph nodes throughout the body.We performed a left submandibular lymph node biopsy,and the results revealed NK/T-cell lymphoma.A biochemical examination indicated Epstein-Barr virus positivity.At the same time,the patient developed hyponatremia.Based on her laboratory examination and clinical manifestation,decreased plasma osmolality,urine osmolality greater than plasma osmolality,lack of skin swelling,normal blood pressure,normal renal function,no adrenal function detected on serology,and no abnormalities in imaging examination of the adrenal glands,the likelihood of SIADH in the patient was high.After fluid restriction and administration of sodium chloride,the patient’s blood sodium level gradually increased.Subsequently,the immune function of the patient declined,there were severe symptoms of infection,and she died of respiratory failure.NK/T-cell lymphoma associated with SIADH has not,to our knowledge,been previously reported in PubMed.This case emphasizes the importance of monitoring serum ion levels,especially serum sodium,in patients with NK/T-cell lymphoma.
文摘A 28-year-old woman with untreated autoimmune disorder, demonstrated skin rash and fever after taking Amoxicillin-clavulanate and developed progressive jaundice. A bone marrow aspiration indicated an increased number of macrophages with hemophagocytosis and liver biopsy showed pure centrilobular cholestasis with necrosis and some absence of portal bile ducts. Furthermore, a serological test for Epstein-Barr virus was positive. Under treatment by liver dialysis and administration of steroids led to rapidly defervescence and clinical improvement. However, liver enzymes were still markedly elevated with persistent anemia, even after immunosuppressive treatment. The patient is currently waiting for liver transplantation. This is the ?rst description of vanishing bile duct syndrome combined with hemophagocytic lymphohistiocytosis, with underlying causes including infection, drug-induced factors and untreated autoimmune disorder.
文摘Hemophagocytic lymphohistiocytosis(HLH)is a cytokine storm syndrome caused by an overactive but ineffective immune reaction.Without prompt diagnosis and treatment,HLH is life-threatening.However,presenting symptoms are often nonspecific,with fatigue and fever being the most common.A high index of suspicion is therefore critical for early diagnosis and timely management.A previously healthy,65-year-old female who initially presented with fever and abdominal pain developed abdominal compartment syndrome(ACS)requiring decompressive laparotomy on hospital day 6.Intraoperative frozen sections of biopsied liver showed intense portal lymphohistiocytic infiltrates.Epstein-Barr virus DNA copy numbers escalated from 600 copies/mL after admission to 134,000 copies/mL before death.The diagnostic criteria of HLH-2004 were met.Patient expired on hospital day 12.It is important to raise awareness of ACS being an unusual presentation of HLH.Recent changes in diagnostic criteria tailored to adult HLH cases are reviewed.
文摘目的:从氨基酸代谢角度探讨乙型肝炎病毒相关慢加急性肝衰竭(HBV-ACLF)湿热瘀黄证和气虚瘀黄证的生物学基础。方法:采用靶向氨基酸代谢组学分析HBV-ACLF湿热瘀黄证组(20例)与气虚瘀黄证组(20例)患者的血清氨基酸代谢谱,寻找两种证型的氨基酸代谢差异特征,分析不同证型中氨基酸代谢与炎症细胞因子表达的相关性。结果:湿热瘀黄证患者体内异亮氨酸表达水平显著高于气虚瘀黄证[(10.36±4.47)μg/ml vs(7.76±4.08)μg/ml,P=0.016],但色氨酸则表达相反[(10.26±4.95)μg/ml vs(14.51±7.32)μg/ml,P=0.014];湿热瘀黄证患者白细胞介素(IL)-6显著高于气虚瘀黄证[10.15(3.88,24.42)pg/ml vs 7.73(3.93,18.83)pg/ml,P=0.017],而IL-2、IL-10显著低于气虚瘀黄证[4.40(2.88,8.55)pg/ml vs 6.60(2.80,15.50)pg/ml,P=0.033;7.19(4.52,15.04)pg/ml vs 8.21(2.19,14.64)pg/ml,P=0.015];多种氨基酸代谢与两种证型的炎症细胞因子表达密切相关,尤其是色氨酸代谢与支链氨基酸代谢;湿热瘀黄证中氨基酸代谢主要与促炎细胞因子IL-2、IL-6相关,按照相关系数大小依次为天冬氨酸、谷氨酸、异亮氨酸、亮氨酸、蛋氨酸、酪氨酸、丝氨酸、赖氨酸、谷氨酰胺、色氨酸、苯丙氨酸;气虚瘀黄证中氨基酸代谢与肿瘤坏死因子α(TNF-α)、IL-2、IL-6、IL-10相关,按照相关系数大小依次为天冬氨酸、甘氨酸、丙氨酸、苏氨酸、色氨酸、丝氨酸。异亮氨酸及色氨酸代谢不仅在两种证型间差异有统计学意义,且差异性关联炎症细胞因子的表达。结论:HBV-ACLF湿热瘀黄证和气虚瘀黄证患者在氨基酸代谢及炎症反应中均具有异质性,且两者之间相互关联。色氨酸以及支链氨基酸代谢可能对HBV-ACLF的免疫炎症反应具有重要调节作用,与湿热瘀黄证和气虚瘀黄证的生物学基础密切相关。
基金supported with a grant form the Nat-ural Science Foundation of Shandong Province of China (ZR2021QC212).
文摘Background:Severe fever with thrombocytopenia syndrome(SFTS)is an emerging hemorrhagic fever caused by a tick-borne bunyavirus SFTSV with case fatality up to 30%.The reactivation of Epstein-Barr virus(EBV)has been proven to occur in individuals with various immune suppression conditions.Methods:Here,we diagnosed 22 SFTSV infected patients with PCR in a hospital in Shandong Province,China in 2020.To understand the consequences of SFTSV infection leading to EBV reactivation,we examined EBV reactivation in SFTSV-infected patients with PCR and RT-PCR.Results:We found that EBV was reactivated in 18.2%(4/22)of SFTS patients,suggesting that EBV reactivation is common in SFTS patients.Compared with SFTS patients without EBV reactivation,SFTS patients with EBV-reactivation had a significantly lower median level of serum albumin(32.45 g/L vs.26.95 g/L,p=0.03)and a significantly higher median number of urine red blood cells(0 cells/μL vs.9 cells/μL,p=0.04).Conclusion:SFTS infection can reactivate EBV in patients,which may make the clinical condition of patients worsen.
基金This study was supported by the grants from Beijing Natural Science Foundation (No. 7072026) and the Scientific Research Program of Beijing Municipal Commission of Education (No. KM200710025023).Acknowledgments: We are grateful to Prof. ZHAORI Ge-tu for a critical reading of the manuscript. We are also grateful to the anonymous peer-reviewers whose constructive comments have significantly improved this paper.
文摘Background Chronic active Epstein-Barr virus infection (CAEBV) has been previously reported to be sometimes associated with an aggressive clinical course. The characteristics of CAEBV in Mainland Chinese pediatric patients are largely unreported. The main aims of this survey were to recognize the clinical features of CAEBV in children and to explore its diagnostic criteria and risk factors. Methods A retrospective study was performed on 53 pediatric patients (36 boys and 17 girls) with CAEBV who were admitted to Beijing Children's Hospital between 2003 and 2007. All their medical records were reviewed and analyzed. For each patient, demographic, clinical, laboratory data and outcome were collected. Independent-samples t test was used for statistical analysis. Results The age at onset of CAEBV was from 2 months to 14.6 years (mean (5.3_+3.3) years). At the time of onset, 43.4% patients had an infectious mononucleosis-like symptom. Most patients exhibited intermittent fever (92.5%, 49/53), hepatomegaly (81.1%, 43/53) and splenomegaly (77.4%, 41/53). Life-threatening complications including hemophagocytic syndrome (24.5%,13/53), interstitial pneumonia (24.5%, 13/53), hepatic failure (15.1%, 8/53) and malignant lymphoma (11.3%, 6/53) were also observed. The serum EBV DNA level in 23 patients with CAEBV was in the range of 5.05×10^2-4.60×10^6 copies/ml with a mean value of 103.7 copies/ml. Many patients with CAEBV generally had continuous symptoms during the observational period. Eleven out of 42 patients (26.2%) died 7 months to 3 years after onset. Deceased patients were more likely to have had lower platelet counts and albumin levels than the living patients (P 〈0.05 for all comparisons). Conclusions The study reveals that CAEBV in Chinese pediatric patients has a severe clinical course and prognosis is poor. Thrombocytopenia and decreases in albumin might potentially be risk factors for a poor prognosis. EBV loads should be measured and tissue should be stained on hybridization probes for EBV-encoded small RNA (EBER) if a patient presents with the known symptoms of CAEBV.
文摘Background This study aimed to evaluate the feasibility and clinical effect of haploidentical hematopoietic stem cell transplantation(haplo-HSCT)for the treatment of pediatric patients with chronic active Epstein-Barr virus infection(CAEBV).Methods Children with CAEBV who did not have matched donors and underwent haplo-HSCT in Beijing Children's Hospital,Capital Medical University,from October 2016 to June 2020 were analyzed retrospectively.Data relating to the clinical manifestations,engraftment,and prognosis of the children were extracted from medical records.Results Twenty-five patients,including 16 males and 9 females,with an onset age of 5.0±2.6 years and a transplantation age of 6.9±2.9 years,were enrolled irnhis study.The mean time from diagnosis to transplantation was 3.8(2.0-40.2)months.The mean observation time was 19.0±12.0 months.Three patients received the reduced intensity conditioning regimen,and the remaining patients all received the modified myeloablative conditioning regimen.By the end of the follow-up,23 patients were characterized by disease-free survival(DFS),22 were characterized by event-free survival(EFS).and two died.One of the patients died of thrombotic microangiopathy(TMA),and another died of graft versus host disease(GVHD);this patient discontinued the treatment for economic reasons.The 3-year overall survival(OS)rate was estimated to be 92.0%±5.4%,and the 3-year EFS rate was estimated to be 87.4%±6.8%.All active patients survived after HSCT event-free.Acute GVHD degrees 1-3 were observed in ten patients(40.0%),and degree IV was observed in six(24.0%),who were all cured except for one patient.Chronic GVHD was observed in nine(36.0%),and most of these cases were mild.The incidence of TMA and veno-occlusive disease(VOD)was 28.0%and 4.0%.Conclusions Haploidentical hematopoietic stem cell transplantation is safe and effective in the treatment of pediatric CAEBV and can be used as an alternative therapy without matched donors or emergency transplantation.Patients with active disease before HSCT also benefited from haplo-HSCT.Haplo-HSCT requires careful monitoring for complications,such as GVHD and TMA.Early detection of TMA and timely treatment can reduce mortality and can improve the survival rate.
基金Beijing Municipal Science&Technology Commission,Grant/Award Number:Z171100001017050National Science and Technology Key Projects,Grant/Award Number:2017ZX09304029。
文摘Importance:Allogeneic hematopoietic stem cell transplantation(alloHSCT)is considered the only effective treatment for chronic active Epstein–Barr virus infection(CAEBV).The clinical efficacy and safety of allo-HSCT with different conditioning regimens in children with CAEBV remain unclear.Objective:To evaluate the effectiveness and safety of allo-HSCT with the modified myeloablative conditioning(MAC)regimen for children with CAEBV and also the factors affecting the outcomes.Methods:We retrospectively analyzed children with CAEBV who underwent allo-HSCT with the modified MAC regimen at Beijing Children’s Hospital,Capital Medical University from October 2016 to June 2021.Data related to the clinical manifestations,engraftment,and outcome were extracted from the medical records.Results:The cohort comprised 41 patients(24 males,17 females)with a median transplantation age of 92.6(60.4,120.7)months and a median follow-up time of 28.2(15.3,40.2)months.Four patients(9.8%)died,among which three died from primary disease relapse,and one died from grade IV acute graft-versus-host diseases(aGVHD)after stopping treatment.The 3-year overall survival(OS)and 3-year event-free survival(EFS)rates were 88.8%±5.4%and 85.0%±5.7%,respectively.The 3-year OS and EFS did not significantly differ between the patients with hemophagocytic lymphohistiocytosis(HLH)and the patient without HLH(87.7%±6.8%vs.91.7%±8.0%,P=0.790;85.0%±6.9%vs.84.6%±10.0%,P=0.921),or among the patients with complete remission,partial remission,and activity disease before HSCT(all P>0.05).Multivariate analysis showed that grade III–IV aGVHD was a risk factor for mortality(Hazards ratio:11.65,95%confidence interval:1.00,136.06;P=0.050).Interpretation:Allo-HSCT with the modified MAC regimen is safe and effective for pediatric CAEBV.This treatment benefits patients with HLH or active disease.Patients with Grade III–IV aGVHD may be associated with worse outcomes.