Document Detail

Cardiac nonamyloidotic immunoglobulin deposition disease.
MedLine Citation:
PMID:  16341150     Owner:  NLM     Status:  MEDLINE    
Cardiac nonamyloidotic immunoglobulin (Ig) deposition disease (CIDD) is a rare disorder characterized by Ig deposition in the myocardium associated with plasma cell dyscrasias. A retrospective review of cardiac biopsies performed at two different institutions identified eight patients with CIDD. All patients had plasma cell dyscrasias with monoclonal gammopathy. Three had IgG lambda, two had IgG kappa, one had IgD kappa and one each had free kappa and free lambda light chain. Four patients had concurrent amyloidosis involving other organs. One had amyloidosis of kidney alone, one had amyloidosis of kidney and abdominal fat pad and two others had amyloidosis of bone marrow vasculature. Three patients had dialysis-dependent renal insufficiency. None of the patients had symptoms of heart failure. Six patients had echocardiographically demonstrable concentric left ventricular hypertrophy with diastolic dysfunction. Two patients had significant cardiac arrhythmias requiring medical intervention. On endomyocardial biopsy, all eight had normal appearing myocardium on light microscopy with negative Congo Red and Thioflavin T stains. On immunofluorescent staining of the cardiac biopsies, all eight stained positive for interstitial Ig deposition. Electron microscopy (EM) confirmed the presence of granular deposits of Igs in the myocardium in five of the eight patients. EM studies were not available in one patient and two others had normal EM studies. In conclusion, CIDD should be considered in the spectrum of cardiovascular pathology in patients with plasma cell dyscrasias. They often, but not always, have left ventricular hypertrophy. These patients may be at risk for developing arrhythmias as well as diastolic dysfunction. Unless immunofluorescent and EM studies are performed routinely in biopsy material, this entity may be missed in the absence of amyloidosis. Concurrent amyloidosis in other organs sheds a unique perspective into the role of local microenvironment in the pathogenesis of systemic Ig deposition disease and amyloidosis.
Amir A Toor; Ben A Ramdane; Jacob Joseph; Maria Thomas; Carl O'Hara; Bart Barlogie; Patrick Walker; Lija Joseph
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc     Volume:  19     ISSN:  0893-3952     ISO Abbreviation:  Mod. Pathol.     Publication Date:  2006 Feb 
Date Detail:
Created Date:  2006-01-20     Completed Date:  2006-03-21     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8806605     Medline TA:  Mod Pathol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  233-7     Citation Subset:  IM    
Myeloma Institute for Research and Therapy, University of Arkansas for Medical Sciences, Little Rock, AR, USA.
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MeSH Terms
Fluorescent Antibody Technique
Heart Diseases / immunology,  pathology,  physiopathology
Immunoglobulin A / immunology
Immunoglobulin G / immunology
Immunoglobulin M / immunology
Immunoglobulin kappa-Chains / immunology
Immunoglobulin lambda-Chains / immunology
Microscopy, Electron
Middle Aged
Myocardium / immunology*,  pathology,  ultrastructure
Paraproteinemias / pathology*
Reg. No./Substance:
0/Immunoglobulin A; 0/Immunoglobulin G; 0/Immunoglobulin M; 0/Immunoglobulin kappa-Chains; 0/Immunoglobulin lambda-Chains

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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