Multiple Myeloma Presenting With CHF

Dyspnea, orthopnea, and weight loss sent a 40-year-old woman for medical consultation. Fifteen years earlier, the patient had been nephrectomized because of left kidney lithiasis. There was no history of other symptoms or diseases.

Drs K. H. Katsanos, Moses S. Elisaf, K. L. Bourantas, and E. V. Tsianos of Ioannina, Greece, noted the woman as thin, with edema of the lower legs, hepatosplenomegaly, and orthostatic hypotension. Laboratory findings showed anemia, thrombocytosis, leukocytosis, and elevated erythrocyte sedimentation rate and lactate dehydrogenase levels.

An echocardiogram revealed severe hypertrophy of the intraventricular septum (IVS) (IVS distance = 1.9 cm) and of the posterior wall of the left ventricle on left parasternal view. The IVS also had a sparkling appearance.

The suspected diagnosis of cardiac amyloidosis was confirmed when perivascular amyloidosis was revealed on Congo red and Cresyl violet stains of tissue from the patient's lip. Subsequent laboratory investigation, including bone radiographs, bone marrow aspirate, serum and urine protein electrophoresis, and immunoelectrophoresis, led to the diagnosis of IgA k-light chain multiple myeloma presenting with congestive heart failure caused by cardiac immunocyte-derived, or AL, amyloidosis.

This patient was treated initially with vincristine, doxorubicin, and dexamethasone; colchicine was added later. She died of heart failure 6 months after diagnosis.