Document Type : Case Report
MSc Student, Student Research Committee AND Department of Cardiac Surgery, Mashhad University of Medical Sciences, Mashhad, Iran
Assistant Professor, Department of Emergency, Dey 9th Hospital, Torbat Heydariyeh University of Medical Sciences, Torbat Heydariyeh, Iran
Department of Obstetrics and Gynecology, Dey 9th Hospital, Torbat Heydariyeh University of Medical Sciences, Torbat Heydariyeh, Iran
Assistant Professor, Department of Pharmacology, Torbat Heydariyeh University of Medical Sciences, Torbat Heydariyeh, Iran
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.
- 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.
- Dubrey SW, Hawkins PN, Falk RH. Amyloid diseases of the heart: Assessment, diagnosis, and referral. Heart 2011; 97(1): 75-84.
- Falk RH. Diagnosis and management of the cardiac amyloidoses. Circulation 2005; 112(13): 2047-60.
- 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.
- Koyama J, Falk RH. Prognostic significance of strain Doppler imaging in light-chain amyloidosis. JACC Cardiovasc Imaging 2010; 3(4): 333-42.
- 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.
- Merlini G, Seldin DC, Gertz MA. Amyloidosis:
- Pathogenesis and new therapeutic options. J Clin Oncol 2011; 29(14): 1924-33.
- 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.
- 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.
- Palladini G, Merlini G. Current treatment of AL amyloidosis. Haematologica 2009; 94(8):
- 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.
- 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.