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J Nephropathol. 2014;3(2): 63-68.
doi: 10.12860/jnp.2014.14
PMID: 24772399
PMCID: PMC3999586
Scopus ID: 84925116105
  Abstract View: 1722
  PDF Download: 788

Case Report

Hypertension, renal failure, and edema in a 38-year-old man: light chain deposition disease; a case report and review of the literature

Sarmad Said 1, Chad J. Cooper 1, Azikiwe C. Nwosu 1, Jorge E. Bilbao 2, German T. Hernandez 1 *

1 Division of Nephrology & Hypertension, Department of Internal Medicine, Paul L. Foster School of Medicine, Texas Tech University Health Sciences Center at El Paso, El Paso, Texas, USA
2 Pathology Professional Services, El Paso, Texas, USA
Corresponding author: German T. Hernandez , Division of Nephrology & Hypertension, Department of Internal Medicine, Paul L. Foster School of Medicine, Texas Tech University Health Sciences Center at El Paso, 4800 Alberta Avenue, El Paso, Texas, USA 79905. german.hernandez@ttuhsc.edu

Abstract

Background: Monoclonal immunoglobulin deposition disease (MIDD) is a rare disease, usually manifesting between the 5th and 6th decades of life but can also occur earlier. Characteristic feature of MIDD is a non-fibrillar, Congo red negative deposition of monoclonal immunoglobulins in various organs, including the kidneys. Depending on the composition of the deposits, MIDD is classified 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 glomerulopathy on light microscopy and diffuse linear staining of glomerular and tubular basement membranes on immunofluorescence (IF) microscopy.

Case presentation: A 38-year-old male patient recently diagnosed with hypertension presented with lower extremity edema, shortness of breath, and fatigue. The workup that was performed in a different hospital 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. Further laboratory workup revealed an increase of IgM, kappa chain and ß2 microglobulin chain, in addition to proteinuria and renal insufficiency. Bone marrow biopsy demonstrated an involvement of 30% with plasma cells. The flow cytometry test showed monotypic plasma cells expressing intracytoplasmic kappa light chain restriction with kappa to lambda ratio of 35/1. The diagnosis of LCDD was established. Treatment with steroids and bortezomib was initiated.

Conclusions: MIDD is an unusual disease and LCDD is the most common form of MIDD. The peak incidence 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 clinical presentation. Nodular sclerosing glomerulopathy is found in about 60% of the affected patients. Early diagnosis and early treatment improve the prognostic course of LCDD.

Keywords: Contrast-induced nephropathy, Contrast media, Percutaneous coronary intervention

Implication for health policy/practice/research/medical education:

Monoclonal immunoglobulin deposition disease (MIDD) is unusual disease and light chain deposition disease (LCDD) is the most common form of MIDD. The peak incidence 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 clinical presentation. Nodular sclerosing glomerulopathy is found in about 60% of the affected patients. Early diagnosis and treatment improve the prognostic course of LCDD.

Please cite this paper as: Said S, Cooper JC, Nwosu AC, Bilbao JE, Hernandez GT. Hypertension, renal failure, and edema in a 38-year-old man: light chain deposition disease; a case report and review of the literature. J Nephropathol. 2014; 3(2): 63-68. DOI: 10.12860/jnp.2014.14

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ePublished: 01 Apr 2014
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