A higher prevalence of immunological processes has recently been reported in patients with hepatitis C virus(HCV)infection,focusing the attention of physicians and researchers on the close association between HCV and ...A higher prevalence of immunological processes has recently been reported in patients with hepatitis C virus(HCV)infection,focusing the attention of physicians and researchers on the close association between HCV and immune disorders.HCV lymphotropism represents the most important step in the pathogenesis of virusrelated immunological diseases and experimental,virologic,and clinical evidence has demonstrated a trigger role for HCV both in systemic autoimmune diseases,such as rheumatoid arthritis,Sj?gren syndrome,hemolytic anemia and severe thrombocytopenia,and in organ-specific autoimmune diseases,such as autoimmune hepatitis,thyroid disorders and diabetes.This review will outline the principal aspects of such HCVinduced immunological alterations,focusing on the prevalence of these less characterized HCV extrahepatic manifestations.展开更多
Background Langerhans cell histiocytosis(LCH)is a group of diseases characterized by the proliferation and accumulation of Langerhans cells.Clinical presentations of LCH vary widely.Data sources A PubMed search was co...Background Langerhans cell histiocytosis(LCH)is a group of diseases characterized by the proliferation and accumulation of Langerhans cells.Clinical presentations of LCH vary widely.Data sources A PubMed search was conducted using Clinical Queries with the key term "Langerhans cell histiocytosis".The search strategy included meta-analyses,randomized controlled trials,clinical trials,observational studies,and reviews.This paper is based on,but not limited to,the search results.Results Generally,patients with LCH can be divided into two groups based on the extent of involvement at diagnosis,namely,single-system LCH and multisystem LCH.The involvement may be unifocal or multifocal.Patients with isolated bone lesions typically present between 5 and 15 years of age,whereas those with multisystem LCH tend to present before 5 years of age.The clinical spectrum is broad,ranging from an asymptomatic isolated skin or bone lesion to a life-threatening multisystem condition.Clinical manifestations include,among others,"punched out" lytic bone lesion,seborrheic dermatitis-like erup-tion,erythematous/reddish-brown crusted/scaly papules/maculopapules/plaques/patches,and eczematous lesions,diabetes insipidus,hepatosplenomegaly,cytopenias,lymphadenopathy,and an acute fulminant disseminated multisystem condition presenting with fever,skin rash,anemia,thrombocytopenia,lymphadenopathy,and hepatosplenomegaly.The diagnosis is clinicopathologic,based on typical clinical findings and histologic/immunohistochemical examination of a biopsy of lesional tissue.Positive CD1a,S100,and/or CD207(Langerin)immunohistochemical staining of lesional cells is required for a definitive diagnosis.Watchful waiting is recommended for patients with skin-only LCH.Patients with symptomatic or refractory skin-only LCH may be treated with topical tacrolimus/corticosteroids,topical nitrogen mustard,oral methotrexate,or oral hydroxyurea.The current recommended first-line therapy for patients with multisystem LCH is 12 months therapy with prednisone and vinblast展开更多
AIM To study the management, complications and outcomes of adult patients admitted with hemophagocytic lymphohistiocytosis(HLH) in the intensive care unit(ICU).METHODS We performed a retrospective observational study ...AIM To study the management, complications and outcomes of adult patients admitted with hemophagocytic lymphohistiocytosis(HLH) in the intensive care unit(ICU).METHODS We performed a retrospective observational study of adult patients with the diagnosis of "HLH" admitted to the two academic medical ICUs of Baylor College of Medicine between 01/01/2013 to 06/30/2017. HLH was diagnosed using the HLH-2004 criteria proposed by the Histiocyte Society.RESULTS Sixteen adult cases of HLH were admitted to the medical ICUs over 4 years.Median age of presentation was 49 years and 10(63%) were males. Median Sequential Organ Failure Assessment(SOFA) score at the time of ICU admission was 10. Median ICU length of stay(LOS) was 11.5 d and median hospital LOS was 29 d. Septic shock and acute respiratory failure accounted for majority of diagnoses necessitating ICU admission. Septic shock was the most common ICU complication seen in(88%) patients, followed by acute kidney injury(81%) and acute respiratory failure requiring mechanical ventilation(75%). Nine patients(56%) developed disseminated intravascular coagulation and eight(50%) had acute liver failure. 10 episodes of clinically significant bleeding were observed.Multi system organ failure was the most common cause of death seen in 12(75%)patients. The 30 d mortality was 37%(6 cases) and 90 d mortality was 81%(13 cases). There was no difference in mortality based on age(above or less than 50 years), SOFA score on ICU admission(more than or less than 10),immunosuppression, time to diagnose HLH or direct ICU admission versus floor transfer.CONCLUSION HLH is a devastating disease associated with poor outcomes in ICU. Intensivists need to have a high degree of clinical suspicion for HLH in patients with septic shock/multi system organ failure and progressive bi/pancytopenia who are not responding to standard management in ICU.展开更多
目的:分析T细胞大颗粒淋巴细胞白血病(T-cell large granular lymphocytic leukemia,T-LGLL)患者的临床特征、治疗方案及生存情况。方法:回顾性分析2009年2月至2019年12月山东大学齐鲁医院收治的21例T-LGLL患者的临床及实验室检查资料,...目的:分析T细胞大颗粒淋巴细胞白血病(T-cell large granular lymphocytic leukemia,T-LGLL)患者的临床特征、治疗方案及生存情况。方法:回顾性分析2009年2月至2019年12月山东大学齐鲁医院收治的21例T-LGLL患者的临床及实验室检查资料,总结其临床特征、治疗方案及预后情况。结果:T-LGLL患者多为中老年男性,主要临床表现为乏力(61.9%)、感染(28.6%)、脾肿大(61.9%),6例患者合并自身免疫性疾病。T-LGLL患者血常规通常表现为白细胞减少(42.9%)和(或)贫血(71.4%),骨髓涂片可见大颗粒淋巴细胞增多,骨髓活检可见粒红系增生受抑,典型的T-LGLL免疫表型为CD3^+CD4^-CD8^+CD57^+CD16^+,88.9%患者TCR基因重排阳性。T-LGLL通常疾病进展缓慢,免疫抑制治疗总有效率为60%,血液学完全缓解率为20%,部分缓解率为40%。结论:T-LGLL为一种以血细胞减少、骨髓及外周血中大颗粒淋巴细胞克隆性增多为特征的疾病,常合并自身免疫性疾病,其疾病进展较慢,一线免疫抑制治疗效果尚可。展开更多
文摘A higher prevalence of immunological processes has recently been reported in patients with hepatitis C virus(HCV)infection,focusing the attention of physicians and researchers on the close association between HCV and immune disorders.HCV lymphotropism represents the most important step in the pathogenesis of virusrelated immunological diseases and experimental,virologic,and clinical evidence has demonstrated a trigger role for HCV both in systemic autoimmune diseases,such as rheumatoid arthritis,Sj?gren syndrome,hemolytic anemia and severe thrombocytopenia,and in organ-specific autoimmune diseases,such as autoimmune hepatitis,thyroid disorders and diabetes.This review will outline the principal aspects of such HCVinduced immunological alterations,focusing on the prevalence of these less characterized HCV extrahepatic manifestations.
文摘Background Langerhans cell histiocytosis(LCH)is a group of diseases characterized by the proliferation and accumulation of Langerhans cells.Clinical presentations of LCH vary widely.Data sources A PubMed search was conducted using Clinical Queries with the key term "Langerhans cell histiocytosis".The search strategy included meta-analyses,randomized controlled trials,clinical trials,observational studies,and reviews.This paper is based on,but not limited to,the search results.Results Generally,patients with LCH can be divided into two groups based on the extent of involvement at diagnosis,namely,single-system LCH and multisystem LCH.The involvement may be unifocal or multifocal.Patients with isolated bone lesions typically present between 5 and 15 years of age,whereas those with multisystem LCH tend to present before 5 years of age.The clinical spectrum is broad,ranging from an asymptomatic isolated skin or bone lesion to a life-threatening multisystem condition.Clinical manifestations include,among others,"punched out" lytic bone lesion,seborrheic dermatitis-like erup-tion,erythematous/reddish-brown crusted/scaly papules/maculopapules/plaques/patches,and eczematous lesions,diabetes insipidus,hepatosplenomegaly,cytopenias,lymphadenopathy,and an acute fulminant disseminated multisystem condition presenting with fever,skin rash,anemia,thrombocytopenia,lymphadenopathy,and hepatosplenomegaly.The diagnosis is clinicopathologic,based on typical clinical findings and histologic/immunohistochemical examination of a biopsy of lesional tissue.Positive CD1a,S100,and/or CD207(Langerin)immunohistochemical staining of lesional cells is required for a definitive diagnosis.Watchful waiting is recommended for patients with skin-only LCH.Patients with symptomatic or refractory skin-only LCH may be treated with topical tacrolimus/corticosteroids,topical nitrogen mustard,oral methotrexate,or oral hydroxyurea.The current recommended first-line therapy for patients with multisystem LCH is 12 months therapy with prednisone and vinblast
文摘AIM To study the management, complications and outcomes of adult patients admitted with hemophagocytic lymphohistiocytosis(HLH) in the intensive care unit(ICU).METHODS We performed a retrospective observational study of adult patients with the diagnosis of "HLH" admitted to the two academic medical ICUs of Baylor College of Medicine between 01/01/2013 to 06/30/2017. HLH was diagnosed using the HLH-2004 criteria proposed by the Histiocyte Society.RESULTS Sixteen adult cases of HLH were admitted to the medical ICUs over 4 years.Median age of presentation was 49 years and 10(63%) were males. Median Sequential Organ Failure Assessment(SOFA) score at the time of ICU admission was 10. Median ICU length of stay(LOS) was 11.5 d and median hospital LOS was 29 d. Septic shock and acute respiratory failure accounted for majority of diagnoses necessitating ICU admission. Septic shock was the most common ICU complication seen in(88%) patients, followed by acute kidney injury(81%) and acute respiratory failure requiring mechanical ventilation(75%). Nine patients(56%) developed disseminated intravascular coagulation and eight(50%) had acute liver failure. 10 episodes of clinically significant bleeding were observed.Multi system organ failure was the most common cause of death seen in 12(75%)patients. The 30 d mortality was 37%(6 cases) and 90 d mortality was 81%(13 cases). There was no difference in mortality based on age(above or less than 50 years), SOFA score on ICU admission(more than or less than 10),immunosuppression, time to diagnose HLH or direct ICU admission versus floor transfer.CONCLUSION HLH is a devastating disease associated with poor outcomes in ICU. Intensivists need to have a high degree of clinical suspicion for HLH in patients with septic shock/multi system organ failure and progressive bi/pancytopenia who are not responding to standard management in ICU.
文摘目的:分析T细胞大颗粒淋巴细胞白血病(T-cell large granular lymphocytic leukemia,T-LGLL)患者的临床特征、治疗方案及生存情况。方法:回顾性分析2009年2月至2019年12月山东大学齐鲁医院收治的21例T-LGLL患者的临床及实验室检查资料,总结其临床特征、治疗方案及预后情况。结果:T-LGLL患者多为中老年男性,主要临床表现为乏力(61.9%)、感染(28.6%)、脾肿大(61.9%),6例患者合并自身免疫性疾病。T-LGLL患者血常规通常表现为白细胞减少(42.9%)和(或)贫血(71.4%),骨髓涂片可见大颗粒淋巴细胞增多,骨髓活检可见粒红系增生受抑,典型的T-LGLL免疫表型为CD3^+CD4^-CD8^+CD57^+CD16^+,88.9%患者TCR基因重排阳性。T-LGLL通常疾病进展缓慢,免疫抑制治疗总有效率为60%,血液学完全缓解率为20%,部分缓解率为40%。结论:T-LGLL为一种以血细胞减少、骨髓及外周血中大颗粒淋巴细胞克隆性增多为特征的疾病,常合并自身免疫性疾病,其疾病进展较慢,一线免疫抑制治疗效果尚可。
