PMID- 11025347
OWN - NLM
STAT- MEDLINE
DCOM- 20001130
LR  - 20171101
IS  - 0008-6312 (Print)
IS  - 0008-6312 (Linking)
VI  - 93
IP  - 4
DP  - 2000
TI  - Infiltrative nonamyloidotic monoclonal immunoglobulin light chain cardiomyopathy: 
      an underappreciated manifestation of plasma cell dyscrasias.
PG  - 220-8
AB  - BACKGROUND: Infiltrative cardiomyopathies are characterized by diastolic 
      dysfunction. In monoclonal plasma cell dyscrasias, organ compromise may be 
      produced by tissue deposition of monoclonal immunoglobulins or their constituent 
      peptides independently of the effects of unbridled plasma cell proliferation. The 
      deposits may be fibrillar, as in light chain amyloid (AL) or nonfibrillar, as in 
      light chain deposition disease (LCDD). AL disease of the heart is a restrictive 
      cardiomyopathy. We hypothesized that, despite differences in physical properties, 
      nonamyloidotic light chain deposition in the myocardium could produce similar 
      clinical and physiological abnormalities. METHODS: Cardiac tissue from five 
      patients with LCDD and cardiac dysfunction was examined by immunohistochemical 
      and electron microscopic techniques. Hospital charts, electrocardiograms, 
      echocardiograms and cardiac catheterization results were reviewed. In two cases, 
      the original echocardiograms were reanalyzed. RESULTS: The five patients with 
      nonamyloidotic light chain deposits in the myocardium had either mechanical or 
      electrocardiographic abnormalities. In four with adequate clinical documentation, 
      the diastolic dysfunction and conduction abnormalities were similar or identical 
      to that described in cardiac AL disease. CONCLUSIONS: Although nonamyloidotic 
      immunoglobulin light chain deposits in the myocardium differ in distribution and 
      ultrastructural organization from the fibrillar deposits of AL disease, an 
      analogous pattern of diastolic dysfunction and conduction disturbances results. 
      The diagnosis should be considered in patients with a plasmacytic dyscrasia and 
      restrictive cardiomyopathy in whom Congo red staining of endomyocardial biopsy 
      tissue is negative. The diagnosis can be established by using the appropriate 
      immunohistochemical and ultrastructural tissue examinations.
CI  - Copyright 2000 S. Karger AG, Basel
FAU - Buxbaum, J N
AU  - Buxbaum JN
AD  - Department of Medicine, New York University School of Medicine and Research 
      Service, New York University Medical Center, New York, NY 10016, USA.
FAU - Genega, E M
AU  - Genega EM
FAU - Lazowski, P
AU  - Lazowski P
FAU - Kumar, A
AU  - Kumar A
FAU - Tunick, P A
AU  - Tunick PA
FAU - Kronzon, I
AU  - Kronzon I
FAU - Gallo, G R
AU  - Gallo GR
LA  - eng
PT  - Comparative Study
PT  - Journal Article
PL  - Switzerland
TA  - Cardiology
JT  - Cardiology
JID - 1266406
RN  - 0 (Immunoglobulin Light Chains)
RN  - 0 (Serum Amyloid P-Component)
SB  - IM
MH  - Adult
MH  - Biopsy
MH  - Cardiac Catheterization
MH  - Cardiomyopathy, Restrictive/*etiology/metabolism
MH  - Diagnosis, Differential
MH  - Echocardiography, Doppler
MH  - Female
MH  - Fluorescent Antibody Technique
MH  - Humans
MH  - Immunoglobulin Light Chains/*metabolism
MH  - Male
MH  - Middle Aged
MH  - Myocardial Contraction
MH  - Myocardium/metabolism/ultrastructure
MH  - Paraproteinemias/*complications/metabolism
MH  - Serum Amyloid P-Component/*metabolism
EDAT- 2000/10/12 11:00
MHDA- 2001/02/28 10:01
CRDT- 2000/10/12 11:00
PHST- 2000/10/12 11:00 [pubmed]
PHST- 2001/02/28 10:01 [medline]
PHST- 2000/10/12 11:00 [entrez]
AID - 7030 [pii]
AID - 10.1159/000007030 [doi]
PST - ppublish
SO  - Cardiology. 2000;93(4):220-8. doi: 10.1159/000007030.