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۱Hypertension, renal failure, and edema in a 38–year–old man: light chain deposition disease; a case report and review of the literature
اطلاعات انتشار: Journal of nephropathology، سوم،شماره۲، Apr ۲۰۱۴، سال
تعداد صفحات: ۶
Background: Monoclonal immunoglobulin deposition disease (MIDD) is a rare disease, usuallymanifesting between the 5th and 6th decades of life but can also occur earlier. Characteristic featureof MIDD is a non–fibrillar, Congo red negative deposition of monoclonal immunoglobulins invarious organs, including the kidneys. Depending on the composition of the deposits, MIDD isclassified into 3 types; light chain deposition disease (LCDD), which is the most common form,heavy chain deposition disease (HCDD) and light and heavy chain deposition disease (LHCDD).Kidney involvement is common in MIDD. Renal biopsy reveals nodular sclerosing glomerulopathyon light microscopy and diffuse linear staining of glomerular and tubular basement membranes onimmunofluorescence (IF) microscopy.Case presentation: A 38–year–old male patient recently diagnosed with hypertension presented withlower extremity edema, shortness of breath, and fatigue. The workup that was performed in a differenthospital prior to this admission, demonstrated the presence of significant proteinuria and renal failure.He was intermittently dialyzed and a renal biopsy was obtained, which showed LCDD. Furtherlaboratory workup revealed an increase of IgM, kappa chain and ß2 microglobulin chain, in additionto proteinuria and renal insufficiency. Bone marrow biopsy demonstrated an involvement of 30%with plasma cells. The flow cytometry test showed monotypic plasma cells expressing intracytoplasmickappa light chain restriction with kappa to lambda ratio of 35\1. The diagnosis of LCDD wasestablished. Treatment with steroids and bortezomib was initiated.Conclusions: MIDD is an unusual disease and LCDD is the most common form of MIDD. The peakincidence is around the 5th and 6th decade of life, however, LCDD can also be found in younger patients.Renal involvement, proteinuria, hematuria, and hypertension are markers of the initial clinicalpresentation. Nodular sclerosing glomerulopathy is found in about 60% of the affectedpatients. Early diagnosis and early treatment improve the prognostic course of LCDD.
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