Infiltrative nonamyloidotic monoclonal immunoglobulin light chain cardiomyopathy: an underappreciated manifestation of plasma cell dyscrasias.
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.
Buxbaum
J N
JN
Department of Medicine, New York University School of Medicine and Research Service, New York University Medical Center, New York, NY 10016, USA.
Genega
E M
EM
Lazowski
P
P
Kumar
A
A
Tunick
P A
PA
Kronzon
I
I
Gallo
G R
GR
D000328:Adult; D001706:Biopsy; D006328:Cardiac Catheterization; D002313:Cardiomyopathy, Restrictive / Q000209:etiology* / Q000378:metabolism; D003937:Diagnosis, Differential; D015150:Echocardiography, Doppler; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D009200:Myocardial Contraction; D009206:Myocardium / Q000378:metabolism / Q000648:ultrastructure; D010265:Paraproteinemias / Q000150:complications* / Q000378:metabolism; D000683:Serum Amyloid P-Component / Q000378:metabolism*
D007147:Immunoglobulin Light Chains; D000683:Serum Amyloid P-Component