The discovery of a strong association between hepatitis C virus(HCV) infection and mixed cryoglobulinemia(MC) has led to an increasingly rare diagnosis of idiopathic essential MC(EMC).The incidence of EMC is high in r...The discovery of a strong association between hepatitis C virus(HCV) infection and mixed cryoglobulinemia(MC) has led to an increasingly rare diagnosis of idiopathic essential MC(EMC).The incidence of EMC is high in regions where there is a comparatively low HCV infection burden and low in areas of high infection prevalence,including HCV.The diagnosis of EMC requires an extensive laboratory investigation to exclude all possible causes of cryoglobulin formation.In addition,although cryoglobulin testing is simple,improper testing conditions will result in false negative results.Here,we present a 46-year-old female patient with a case of EMC with dermatological and renal manifestations,highlighting the importance of extensive investigation to reacha proper diagnosis.We review the need for appropriate laboratory testing,which is often neglected in clinical practice and which can result in false negative results.This review also emphasizes the significance of an extended testing repertoire necessary for better patient management.Despite a strong association of MC with HCV infection and other causes that lead to cryoglobulin formation,EMC remains a separate entity.Correct diagnosis requires proper temperature regulation during sample handling,as well as characterization and quantification of the cryoprecipitate.Inclusion of rheumatoid factor activity and complement levels in the cryoglobulin test-panel promotes better patient management and monitoring.Consensus guidelines should be developed and implemented for cryoglobulin detection and the diagnosis of cryoglobulinemic syndrome,which will reduce variability in inter-laboratory reporting.展开更多
Objective:Cryoglobulinemia often causes systemic vasculitis,thereby damaging to skin and internal organs including kidneys,even life-threatening.This review aimed to introduce the advances in understanding,detection,a...Objective:Cryoglobulinemia often causes systemic vasculitis,thereby damaging to skin and internal organs including kidneys,even life-threatening.This review aimed to introduce the advances in understanding,detection,and treatment of this disease in recent years,with a particular concern to clinical practice.Data sources:All the data in this review were from the English or Chinese literature in the PubMed and China National Knowledge Infrastructure databases as of March 2019.Study selection:This review selected important original articles,meaningful reviews,and some reports on cryoglobulinemia published in recent years and in history,as well as the guidelines for treatment of underlying diseases which lead to cryoglobulinemia.Results:Diagnosis of cryoglobulinemia relies on serum cryoglobulin test,in which to ensure that the blood sample temperature is not less than 37℃ in the entire pre-analysis phase is the key to avoid false negative results.Cryoglobulinemic vasculitis (Cryo Vas),including cryoglobulinemic glomerulonephritis (Cryo GN),usually occurs in types Ⅱ and Ⅲ mixed cryoglobulinemia,and can also be seen in type Ⅰ cryoglobulinemia caused by monoclonal IgG3 or IgG1.Skin purpura,positive serum rheumatoid factor,and decreased serum levels of C4 and C3 are important clues for prompting types Ⅱ and Ⅲ Cryo Vas.Renal biopsy is an important means for diagnosis of Cryo GN,while membranous proliferative GN is the most common pathological type of Cryo GN.In recent years,great advances have been made in the treatment of Cryo Vas and its underlying diseases,and this review has briefly introduced these advances.Conclusions:Laboratory examinations of serum cryoglobulins urgently need standardization.The recent advances in the diagnosis and treatment of Cryo Vas and GN need to be popularized among the clinicians in related disciplines.展开更多
文摘The discovery of a strong association between hepatitis C virus(HCV) infection and mixed cryoglobulinemia(MC) has led to an increasingly rare diagnosis of idiopathic essential MC(EMC).The incidence of EMC is high in regions where there is a comparatively low HCV infection burden and low in areas of high infection prevalence,including HCV.The diagnosis of EMC requires an extensive laboratory investigation to exclude all possible causes of cryoglobulin formation.In addition,although cryoglobulin testing is simple,improper testing conditions will result in false negative results.Here,we present a 46-year-old female patient with a case of EMC with dermatological and renal manifestations,highlighting the importance of extensive investigation to reacha proper diagnosis.We review the need for appropriate laboratory testing,which is often neglected in clinical practice and which can result in false negative results.This review also emphasizes the significance of an extended testing repertoire necessary for better patient management.Despite a strong association of MC with HCV infection and other causes that lead to cryoglobulin formation,EMC remains a separate entity.Correct diagnosis requires proper temperature regulation during sample handling,as well as characterization and quantification of the cryoprecipitate.Inclusion of rheumatoid factor activity and complement levels in the cryoglobulin test-panel promotes better patient management and monitoring.Consensus guidelines should be developed and implemented for cryoglobulin detection and the diagnosis of cryoglobulinemic syndrome,which will reduce variability in inter-laboratory reporting.
文摘Objective:Cryoglobulinemia often causes systemic vasculitis,thereby damaging to skin and internal organs including kidneys,even life-threatening.This review aimed to introduce the advances in understanding,detection,and treatment of this disease in recent years,with a particular concern to clinical practice.Data sources:All the data in this review were from the English or Chinese literature in the PubMed and China National Knowledge Infrastructure databases as of March 2019.Study selection:This review selected important original articles,meaningful reviews,and some reports on cryoglobulinemia published in recent years and in history,as well as the guidelines for treatment of underlying diseases which lead to cryoglobulinemia.Results:Diagnosis of cryoglobulinemia relies on serum cryoglobulin test,in which to ensure that the blood sample temperature is not less than 37℃ in the entire pre-analysis phase is the key to avoid false negative results.Cryoglobulinemic vasculitis (Cryo Vas),including cryoglobulinemic glomerulonephritis (Cryo GN),usually occurs in types Ⅱ and Ⅲ mixed cryoglobulinemia,and can also be seen in type Ⅰ cryoglobulinemia caused by monoclonal IgG3 or IgG1.Skin purpura,positive serum rheumatoid factor,and decreased serum levels of C4 and C3 are important clues for prompting types Ⅱ and Ⅲ Cryo Vas.Renal biopsy is an important means for diagnosis of Cryo GN,while membranous proliferative GN is the most common pathological type of Cryo GN.In recent years,great advances have been made in the treatment of Cryo Vas and its underlying diseases,and this review has briefly introduced these advances.Conclusions:Laboratory examinations of serum cryoglobulins urgently need standardization.The recent advances in the diagnosis and treatment of Cryo Vas and GN need to be popularized among the clinicians in related disciplines.
