PMID- 24772399
OWN - NLM
STAT- PubMed-not-MEDLINE
DCOM- 20140428
LR  - 20220331
IS  - 2251-8363 (Print)
IS  - 2251-8819 (Electronic)
IS  - 2251-8363 (Linking)
VI  - 3
IP  - 2
DP  - 2014
TI  - Hypertension, renal failure, and edema in a 38-year-old man: light chain 
      deposition disease; a case report and review of the literature.
PG  - 63-8
LID - 10.12860/jnp.2014.14 [doi]
AB  - BACKGROUND: Monoclonal immunoglobulin deposition disease (MIDD) is a rare 
      disease, usually manifesting between the 5(th) and 6(th) 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 5(th) and 6(th) 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.
FAU - Said, Sarmad
AU  - Said S
AD  - 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.
FAU - J Cooper, Chad
AU  - J Cooper C
AD  - 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.
FAU - C Nwosu, Azikiwe
AU  - C Nwosu A
AD  - 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.
FAU - E Bilbao, Jorge
AU  - E Bilbao J
AD  - Pathology Professional Services, El Paso, Texas, USA.
FAU - T Hernandez, German
AU  - T Hernandez G
AD  - 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.
LA  - eng
PT  - Case Reports
DEP - 20140401
PL  - Iran
TA  - J Nephropathol
JT  - Journal of nephropathology
JID - 101611182
PMC - PMC3999586
OTO - NOTNLM
OT  - Contrast media
OT  - Contrast-induced nephropathy
OT  - Percutaneous coronary intervention
EDAT- 2014/04/29 06:00
MHDA- 2014/04/29 06:01
PMCR- 2014/01/01
CRDT- 2014/04/29 06:00
PHST- 2013/12/01 00:00 [received]
PHST- 2014/02/02 00:00 [accepted]
PHST- 2014/04/29 06:00 [entrez]
PHST- 2014/04/29 06:00 [pubmed]
PHST- 2014/04/29 06:01 [medline]
PHST- 2014/01/01 00:00 [pmc-release]
AID - 10.12860/jnp.2014.14 [doi]
PST - ppublish
SO  - J Nephropathol. 2014;3(2):63-8. doi: 10.12860/jnp.2014.14. Epub 2014 Apr 1.