There are three subtypes of monoclonal immunoglobulin deposition disease: light chain deposition disease (LCDD), light and heavy chain deposition disease (LHCDD), and heavy chain deposition disease (HCDD). Although it has been considered that LHCDD is a variant of LCDD, information on clinicopathological features and prognosis in LHCDD is presently limited.
We reviewed 5,443 renal biopsies, and evaluated clinicopathological features and outcomes in patients with LHCDD, in comparison with those in patients with LCDD and previously reported patients with HCDD. We also characterized paraprotein deposits in patients with LHCDD.
We identified 6 patients with LHCDD, 6 patients with LCDD, and 1 patient with HCDD. The most common clinicopathological findings in patients with LHCDD were proteinuria, renal insufficiency, and nodular sclerosing glomerulopathy. Three patients had IgG-k deposits and 3 patients had IgG-l deposits. Heavy chain subclass analysis performed in 4 patients showed IgG3 deposits in all patients. Dual immunostaining revealed glomerular colocalization of light and heavy chains. In contrast with LCDD, glomerular C3 and C1q deposits were common findings in LHCDD and HCDD. All patients with LHCDD were treated with steroids and cytotoxic agents, but no effect on proteinuria was observed. Three patients developed end-stage renal disease requiring hemodialysis. The underlying hematological disorders in LHCDD and HCDD were milder than in LCDD. Early renal survival and overall patient survival in our patients appeared to be better in LHCDD than in LCDD.
There are apparent differences in clinicopathological features and prognosis between LHCDD and LCDD. LHCDD is probably more similar to HCDD.
[Show abstract][Hide abstract] ABSTRACT: The biochemical characteristics of 1 37 strains of Citrobacter diversus (Burkey) Werkman and Gillen were determined, and the resulting data are summarized. Members of this species produced indole and ornithine decarboxylase and fermented adonitol but failed to grow in KCN medium. Detectable amounts of hydrogen sulfide were not produced in triple sugar-iron-agar medium but were formed by some strains in peptone-iron-agar. The nomenclature and taxonomic position of these bacteria are discussed, and information that should be helpful for their differentiation from Citrobacter freundii and Enterobacter cloacae is included. Strain 3613-63 (ATCC 27 156) is designated as the neotype strain and is described herein.
International journal of systematic bacteriology 01/1972; 22(1). DOI:10.1099/00207713-22-1-12 · 2.27 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) associated with membranoproliferative features is an extremely rare entity. Information on clinicopathological features and prognosis in this entity is limited.
We reviewed 5,443 renal biopsies processed at our department, and identified 4 patients with PGNMID associated with membranoproliferative features. We evaluated clinicopathological features and outcomes in these patients, and characterized paraprotein deposits by immunofluorescence studies.
Three out of 4 patients had nephrotic syndrome with renal insufficiency at presentation. Cryoglobulin or monoclonal protein in serum and urine was not detected. Renal biopsy showed membranoproliferative features with or without nodular formation. Tubulointerstitial and vascular alterations were mild in three patients. All patients had glomerular IgG-kappa deposits. Heavy chain subclass analysis performed in 3 patients showed IgG3 deposits. Immunofluorescence studies using antibodies specific for gamma-heavy chain C(H)1, C(H)2, and C(H)3 domains and gamma3 hinge did not show any apparent deletion. Confocal microscopy revealed glomerular colocalization of light and heavy chains. On electron microscopy, granular deposits were predominantly mesangial and subendothelial. All patients were treated with steroids and cytotoxic agents, but no effect on proteinuria was observed. The renal outcome was progressive in all patients. Early death was observed in two elder patients. No patient had overt myeloma or lymphoma at presentation or over the course of follow-up (mean 43 months).
Our study suggests a predominance of IgG3-kappa glomerular deposits of nondeleted whole immunoglobulin molecules in PGNMID associated with membranoproliferative features. The clinical outcome in patients with this entity appears to be poor.
[Show abstract][Hide abstract] ABSTRACT: A 44-year-old female was diagnosed with proteinuria due to nodular glomerulosclerosis secondary to light chain deposition disease (LCDD). After 6 years, deterioration of kidney function occurred and autologous stem cell transplantation was considered, but the patient refused specific therapies. The disease progressed slowly, over a period of 8 years reaching now chronic renal insufficiency stage 4 with a creatinine clearance of 20 ml/min, in spite of no specific therapy. This case, documented by repeated biopsies, demonstrates the very slow loss of kidney function, suggesting the possibility of conservative treatment strategies without taking the risks of chemotherapy or autologous stem cell transplantation, since no long term follow up data of these therapies are available for LCDD.
Data provided are for informational purposes only. Although carefully collected, accuracy cannot be guaranteed. The impact factor represents a rough estimation of the journal's impact factor and does not reflect the actual current impact factor. Publisher conditions are provided by RoMEO. Differing provisions from the publisher's actual policy or licence agreement may be applicable.