Authors

• Mateja Sabol — Čakovec Couty Hospital, Čakovec, Croatia
• Pušić — Čakovec Couty Hospital, Čakovec, Croatia — ORCID: 0000-0003-4895-0681
• Martina Magdalenić — Čakovec Couty Hospital, Čakovec, Croatia — ORCID: 0000-0002-9790-449X
• Branko Ostrički — Čakovec Couty Hospital, Čakovec, Croatia — ORCID: 0000-0001-8682-5158
• Mihajlo Kovačić — Čakovec Couty Hospital, Čakovec, Croatia — ORCID: 0000-0002-2577-9474

Keywords

amyloidosis, heart transplantation, heart failure

DOI

Full Text

Systemic amyloidosis is a rare disease caused by the extracellular deposition of amyloid fibrils in various organs and tissues, most commonly in the heart and kidneys. Cardiac involvement is associated with an increased mortality and morbidity, especially in primary light chain (AL) amyloidosis. The treatment of cardiac amyloidosis is twofold, oriented both on the underlying disease and heart failure with possible arrhythmic events. (1-3) We present a case of 61-year-old male patient with lambda light chains multiple myeloma, and heart and kidney AL amyloidosis. Our patient achieved hematologic remission after the VAD chemotherapeutic protocol, but because of Mayo III grade heart amyloidosis he was ineligible for autologous transplantation of peripheral blood stem cells (ASCT). We considered the possibility of heart transplantation, followed by ASCT, which has been described in the literature as good solution in selected cases of advanced cardiac amyloidosis, but the patient was not accepted for the procedure because he did not meet the criteria stated in the guidelines. With mostly empirical medicamentous therapy for restrictive cardiomyopathy and chronic cardiorenal syndrome, and treating many infectious complications he achieved survival of more than 2 years, which is a relatively good result, given the stage of the disease. Cardiac AL amyloidosis is a very rare condition, often diagnosed with delay. The treatment mainstay is to control the plasma cell clone which is producing the light chains, and in suitable patients the autologous transplantation of peripheral blood stem cells represents the treatment of choice. Although controversial, a heart transplantation followed by ASCT is the only option for improving prognosis in younger, eligible patients with severe cardiac AL amyloidosis. The therapy targeting amyloid deposits in tissues is for now only in experimental stages and is not yet applied in clinical practice.

Literature

1. Banypersad SM, Moon JC, Whelan C, Hawkins PN, Wechalekar AD. Updates in cardiac amyloidosis: a review. J Am Heart Assoc. 2012 Apr;1(2):e000364. https://doi.org/10.1161/JAHA.111.000364
2. Falk RH, Alexander KM, Liao R, Dorbala SAL. (Light-Chain) Cardiac Amyloidosis: A Review of Diagnosis and Therapy. J Am Coll Cardiol. 2016;68(12):1323–41. https://doi.org/10.1016/j.jacc.2016.06.053
3. Grogan M, Gertz M, McCurdy A, Roeker L, Kyle R, Kushwaha S, et al. Long term outcomes of cardiac transplant for immunoglobulin light chain amyloidosis: The Mayo Clinic experience. World J Transplant. 2016;6(2):380–8. https://doi.org/10.5500/wjt.v6.i2.380