An unusual clinical course of cardiac Ig deposition disease.

摘要

The presence of monoclonal Ig-associated deposition disease (IgDD), such as primary amyloidosis (AL), as a cause of cardiac failure is considered to be a contraindication for cardiac transplantation because the underlying plasma-cell dyscrasia might eventually affect the donor heart. Here we report on a patient in whom the presence of IgDD was retrospectively recognized as the cause of three successively failing hearts including two allografts. Blood progenitor-cell-supported high-dose mel-phalan therapy fully restored the function of the second transplanted heart, although the serum concentration of the presumably tissue-damaging Ig was only modestly reduced. This case challenges current medical practice of denying patients with IgDD access to cardiac transplants, as the application of high-dose chemotherapy, aimed at reducing the offending Ig-secreting plasma-cell clone, proved successful in restoring normal function and seemingly eliminating IgDD infiltration in the second cardiac graft.