A case of cardiac dysfunction associated with monoclonal gammopathy of undetermined significance

Abstract

The monoclonal gammopathies (MG) are monoclonal neoplasms related to each other by virtue of their development from common progenitors in the B lymphocyte lineage. Cardiac dysfunction in patients with MG is not well established. We experienced a case of cardiac dysfunction associated with MG identified by echocardiography and biopsy. Fifty nine year-old man was admitted because of dyspnea for several months. Echocardiography revealed diastolic dysfunction showing restrictive physiology with elevated left ventricular filling pressure. Bone marrow (BM) studies and immunoelectrophoresis were compatible with monoclonal gammopathy of undetermined significance. Endomyocardial, BM, and enteral biopsies for ruling out for amyloidosis (Congo-red stain) were negative. This is the case of non-amyloidotic light chain deposition cardiomyopathy.

Keywords: Echocardiography; Paraproteinemias; Ventricular Dysfunction.

Fig. 1

In serum (left) and urine (right) immunoelectrophoresis study, lambda type monoclonal gammopathy was shown.

Fig. 2

Electrocardiogram demonstrated regular sinus rhythm with premature atrial beat, left axis deviation, and poor R progression on precordial leads.

Fig. 3

Echocardiography demonstrated increased thickness of the left ventricle (LV) wall and both atrial enlargement and normal systolic function with normal wall motion. Mitral inflow and mitral anular Doppler tissue velocities showed grade 3 diastolic dysfunction and high E/E', suggesting markedly elevated LV filling pressure.

Fig. 4

In bone marrow biopsy, plasma cells were increased and counted up to 6.8% of absolute neutrophil count. (H&E stain, ×200).

Fig. 5

(A) Right ventricular endomyocardial biopsy with Congo-red stain did not show apple-green birefringence in polarized light (×200). (B) Same specimen of immunostaining for lambda light chain showed positive for perivascular deposition of immunoglobulins (brown color, ×400).