Cover Image: Resolution of cardiac amyloidosis after autologous blood stem cell transplantation
Wilfried Agnes Mullens, Lieven Herbots, and Johan Vanhaecke
UZ Gasthuisberg, Leuven, Belgium
A 49-year-old lady was diagnosed with a monoclonal gammopathy of unknown significance (IgG Kappa) in 1993. Subsequently in May 2001, she was diagnosed with biopsy proven primary cardiac amyloidosis presenting as a restrictive cardiomyopathy with heart failure. Initial bone marrow plasmocytosis was 7% and immunofixation on urine showed a monoclonal M-peak consisting of IgG kappa. Pulsed-dose dexamethasone was given in monotherapy without any improvement of symptoms or echocardiographic findings. Following high-dose melphalan treatment (200 mg/m2) and an autologous peripheral blood stem cell transplantation in January 2002, a dramatic clinical improvement was noticed, coinciding with an impressive resolution of the stigmata of amyloidosis on echocardiography and endomyocardial biopsy. Aggressive treatment of the haematological disorder can result in a cure of cardiac amyloidosis.
Figure legend
Echocardiogram and pulsed wave doppler signal of the mitral valve inflow from the apical four chamber view and endomyocardial biopsy using congo red stain before (left panel) and 1 year after stem-cell transplantation (right panel) shows the impressive resolution of the stigmata of amyloidosis. The sparkling appearance of the myocardium, thickening of the ventricular wall, the restrictive inflow pattern of the left ventricle, the pleural effusion, and the massive amyloid deposits all disappeared almost completely 1 year after stem-cell transplantation.
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