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
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:分析EB病毒相关性噬血细胞综合征(EB virus-associated hemophagocytic syndrome,EBVAHS)患儿EB病毒潜伏膜蛋白1(latent membrane protein 1,LMP1)、辅助性T细胞1(helper T cell1,Th1)细胞因子的表达水平及其与疗效的关系。方法:...目的:分析EB病毒相关性噬血细胞综合征(EB virus-associated hemophagocytic syndrome,EBVAHS)患儿EB病毒潜伏膜蛋白1(latent membrane protein 1,LMP1)、辅助性T细胞1(helper T cell1,Th1)细胞因子的表达水平及其与疗效的关系。方法:抽取郑州大学附属儿童医院儿科2014年2月至2018年1月收治的116例EB病毒感染患儿为研究对象,分为单核细胞增多症(infect iousmononucleosis,IM)组与EBV-AHS组,其中IM 66例,EBV-AHS 50例。EBV-AHS组按照国际组织细胞协会推荐的HLH-2004方案给予治疗,于治疗4周后评定近期疗效。另选取同期体检的健康儿童30例作为对照组。对照组于体检时、IM组于确诊时、EBV-AHS组于治疗前、治疗后采血,检测血清LMP1抗体表达及Th1细胞因子水平。结果:与对照组相比,IM组和EBV-AHS组患儿确诊时血清LMP1抗体、白介素(IL)-2、干扰素(IFN)-γ及肿瘤坏死因子(TNF)-α水平显著增高,差异有统计学意义(P<0.05)。IM组和EBV-AHS组患儿血清LMP1抗体,IL-2,IFN-γ,TNF-α水平差异亦有统计学意义(P<0.05)。治疗后EBV-AHS组50例患儿中,疾病缓解者17例(34.00%),有效者22例(44.00%),余下11例(22.00%)疾病活动。疾病缓解者、有效者血清LMP1抗体,IL-2,IFN-γ,TNF-α水平明显较治疗前降低(P<0.05),而疾病活动者血清LMP1抗体,IL-2,IFN-γ,TNF-α水平较治疗前无明显改善(P>0.05);且疾病缓解者、有效者、疾病活动者血清LMP1抗体,IL-2,IFN-γ,TNF-α水平均依次升高,差异有统计学意义(P<0.05)。结论:EBV-AHS患儿血清LMP1抗体表达及Th1细胞因子水平较IM患儿及正常儿童显著升高,且其与疗效密切相关。展开更多
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.
