Document Type : Case Report


1 MSc Student, Student Research Committee AND Department of Cardiac Surgery, Mashhad University of Medical Sciences, Mashhad, Iran

2 Assistant Professor, Department of Emergency, Dey 9th Hospital, Torbat Heydariyeh University of Medical Sciences, Torbat Heydariyeh, Iran

3 Department of Obstetrics and Gynecology, Dey 9th Hospital, Torbat Heydariyeh University of Medical Sciences, Torbat Heydariyeh, Iran

4 Assistant Professor, Department of Pharmacology, Torbat Heydariyeh University of Medical Sciences, Torbat Heydariyeh, Iran

5 Assistant Professor, Department of Cardiology, Torbat Heydariyeh University of Medical Sciences, Torbat Heydariyeh, Iran


BACKGROUND: Amyloidosis is a severe systemic disorder produces by the accumulation of inappropriately amyloid deposition in tissues. Cardiac involvement, as a main type of amyloidosis, has a major impact on prognosis. We describe a biopsy-proven cardiac amyloidosis in an old man with unexpected presentation.CASE REPORT: A 70-year-old man, with a complaint of severe weakness, lightheadedness, and lower limb paresthesia, was admitted to the emergency department. Electrocardiography revealed right bundle branch block and Trifascicular block. Echocardiography study showed a moderately increased thickness of left ventricular wall with concentric pattern as well. Laboratory investigations including serum and urine electrophoresis, and serum free light chain examination as immunofixation assay revealed that κ chains predominated over λ chains in a ratio of 3:2. Our patient with final diagnosis of amyloid light-chain (AL) amyloidosis underwent chemotherapy with melphalan combined with high-dose dexamethasone, CPHPC and monoclonal antibodies for 2 weeks.CONCLUSION: It shows that rapid diagnosis of AL amyloidosis can enhance the prognosis. Applying an optimal strategy for the treatment leads to effective therapy, too.


  1. Bunker D, Gorevic P. AA amyloidosis: Mount Sinai experience, 1997-2012. Mount Sinai Journal of Medicine: A Journal of Translational and Personalized Medicine 2012; 79(6): 749-56.
  2. Dubrey SW, Hawkins PN, Falk RH. Amyloid diseases of the heart: Assessment, diagnosis, and referral. Heart 2011; 97(1): 75-84.
  3. Falk RH. Diagnosis and management of the cardiac amyloidoses. Circulation 2005; 112(13): 2047-60.
  4. Fikrle M, Palecek T, Kuchynka P, Nemecek E, Bauerova L, Straub J, et al. Cardiac amyloidosis: A comprehensive review. Cor et Vasa 2013; 55(1): e60-e75.
  5. Koyama J, Falk RH. Prognostic significance of strain Doppler imaging in light-chain amyloidosis. JACC Cardiovasc Imaging 2010; 3(4): 333-42.
  6. Palladini G, Lavatelli F, Russo P, Perlini S, Perfetti V, Bosoni T, et al. Circulating amyloidogenic free light chains and serum N-terminal natriuretic peptide type B decrease simultaneously in association with improvement of survival in AL. Blood 2006; 107(10): 3854-8.
  7. Merlini G, Seldin DC, Gertz MA. Amyloidosis:
  8. Pathogenesis and new therapeutic options. J Clin Oncol 2011; 29(14): 1924-33.
  9. Mohty D, Damy T, Cosnay P, Echahidi N, Casset-Senon D, Virot P, et al. Cardiac amyloidosis: Updates in diagnosis and management. Arch Cardiovasc Dis 2013; 106(10): 528-40.
  10. Nordlinger M, Magnani B, Skinner M, Falk RH. Is elevated plasma B-natriuretic peptide in amyloidosis simply a function of the presence of heart failure? Am J Cardiol 2005; 96(7): 982-4.
  11. Palladini G, Merlini G. Current treatment of AL amyloidosis. Haematologica 2009; 94(8):
  12. -8.
  13. Reyners AK, Hazenberg BP, Reitsma WD, Smit AJ. Heart rate variability as a predictor of mortality in patients with AA and AL amyloidosis. Eur Heart J 2002; 23(2): 157-61.
  14. Selvanayagam JB, Hawkins PN, Paul B, Myerson SG, Neubauer S. Evaluation and management of the cardiac amyloidosis. J Am Coll Cardiol 2007; 50(22): 2101-10.