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
The evidence in the medical literature on the efficacy and safety of rituximab therapy for primary glomerulonephritis is limited and controversial. We describe two male Caucasian patients with rapidly progressive kidn...The evidence in the medical literature on the efficacy and safety of rituximab therapy for primary glomerulonephritis is limited and controversial. We describe two male Caucasian patients with rapidly progressive kidney failure due to primary proliferative glomerulonephritis. Both of them received high-dose intravenous corticosteroids and oral cyclophosphamide with limited benefit. The first patient(hepatitis C virus-negative mixed cryoglobulinemia) underwent plasma-exchange with intravenous immunoglobulins; he showed significant benefit on kidney function(he became dialysis independent with serum creatinine going back to 1.6 mg/d L) after one rituximab pulse even if urinary abnormalities were still present. No improvement in renal function or urinary changes occurred in the second patient. Both these individuals developed sepsis over the follow-up, the first patient died two months after rituximab therapy. This report is in keeping with the occurrence of severe infections after rituximab therapy in patients with renal impairment at baseline and concomitant high-dose steroids.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The evidence in the medical literature on the efficacy and safety of rituximab therapy for primary glomerulonephritis is limited and controversial. We describe two male Caucasian patients with rapidly progressive kidney failure due to primary proliferative glomerulonephritis. Both of them received high-dose intravenous corticosteroids and oral cyclophosphamide with limited benefit. The first patient(hepatitis C virus-negative mixed cryoglobulinemia) underwent plasma-exchange with intravenous immunoglobulins; he showed significant benefit on kidney function(he became dialysis independent with serum creatinine going back to 1.6 mg/d L) after one rituximab pulse even if urinary abnormalities were still present. No improvement in renal function or urinary changes occurred in the second patient. Both these individuals developed sepsis over the follow-up, the first patient died two months after rituximab therapy. This report is in keeping with the occurrence of severe infections after rituximab therapy in patients with renal impairment at baseline and concomitant high-dose steroids.
