Tags

Type your tag names separated by a space and hit enter

Cardiac nonamyloidotic immunoglobulin deposition disease.
Mod Pathol. 2006 Feb; 19(2):233-7.MP

Abstract

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.

Authors+Show Affiliations

Myeloma Institute for Research and Therapy, University of Arkansas for Medical Sciences, Little Rock, AR, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

16341150

Citation

Toor, Amir A., et al. "Cardiac Nonamyloidotic Immunoglobulin Deposition Disease." Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc, vol. 19, no. 2, 2006, pp. 233-7.
Toor AA, Ramdane BA, Joseph J, et al. Cardiac nonamyloidotic immunoglobulin deposition disease. Mod Pathol. 2006;19(2):233-7.
Toor, A. A., Ramdane, B. A., Joseph, J., Thomas, M., O'Hara, C., Barlogie, B., Walker, P., & Joseph, L. (2006). Cardiac nonamyloidotic immunoglobulin deposition disease. Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc, 19(2), 233-7.
Toor AA, et al. Cardiac Nonamyloidotic Immunoglobulin Deposition Disease. Mod Pathol. 2006;19(2):233-7. PubMed PMID: 16341150.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Cardiac nonamyloidotic immunoglobulin deposition disease. AU - Toor,Amir A, AU - Ramdane,Ben A, AU - Joseph,Jacob, AU - Thomas,Maria, AU - O'Hara,Carl, AU - Barlogie,Bart, AU - Walker,Patrick, AU - Joseph,Lija, PY - 2005/12/13/pubmed PY - 2006/3/22/medline PY - 2005/12/13/entrez SP - 233 EP - 7 JF - Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc JO - Mod. Pathol. VL - 19 IS - 2 N2 - 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. SN - 0893-3952 UR - https://www.unboundmedicine.com/medline/citation/16341150/Cardiac_nonamyloidotic_immunoglobulin_deposition_disease_ L2 - http://dx.doi.org/10.1038/modpathol.3800524 DB - PRIME DP - Unbound Medicine ER -