Hesna BEKTAŞ, Esra DEMİR, Oğuzhan KURŞUN

Acute ischemic stroke patient with cardiac involvement of light chain amyloidosis

Light chain amyloidosis is one of the clonal plasma cell disorders characterized by the accumulation of misfolded light chain in tissues and organs. It may affect many organs and tissues like kidney, heart, nervous system, intestinal systems. Symptoms may mimic several diseases. The natural history of the disease, involvement of other organs and treatment options vary significantly based on the origin of the protein. In amyloid light-chain (AL) amyloidosis, amyloid protein is derived from immunoglobulin light chains and most often involves the kidneys and the heart. Cardiac amyloidosis is important in terms of causing intracardiac thrombus and increases the risk of thromboembolic complications as stroke. We discussed a 55-year-old patient with known cardiac involvement of systemic amyloidosis who presented to an emergency department with loss of consciousness and progressive left-sided weakness and had the diagnosis of acute ischemic stroke.

PDF

___

1. Coban O, Ekizoglu E. Definitions, Classification, Epidemiology and Risk Factors In Brain Vascular Diseases. In: Oge E, ,Baykan B, editors. Text book of Neurology, 2 nd edition. Istanbul; Gunes Medical Bookstore 2015;235-45.
2. Cavusoglu Y, Ozpelit E, Celik A, et.al. Cardiac amyloidosis: Innovations in diagnosis and treatment cardiac amyloidosis. Turkısh Society of Cardiology Ars 2019;2:1-34
3. Ozsan GH. Non-myeloma plasma cell. Turkish J Hematol 2013;3:1.
4. Merlini G, Seldin DC, Gertz MA. Amyloidosis: pathogenesis and new therapeutic options. J Clin Oncol 2011;29:1924-33.
5. Skinner M, Anderson J, Simms R, et al. Treatment of 100 patients with primary amyloidosis: A randomized trial of melphalan, prednisone, and colchicine versus colchicine only. Am J Med 1996;100: 290–8.
6. Schwarz MI. Miscellanous interstitial lung disease. In: Schwarz MI, Jr. King TE, eds. Interstitial Lung Disease. London: B.C Decker Inc. 1998:697-750.
7. Celebi ZK, Kiremitci S, Ozturk B, et al. Secondary amyloıdosıs wıth renal ınvolvement: Histopathological findings and clinical correlation. Turkish J Nephrol 2015;1:21.
8. Zubkov AY, Rabinstein, AA., Dispenzieri, A, et.al. Primary systemic amyloidosis with ischemic stroke as a presenting complication. 2007;69:11:1136-41.
9. Kyle RA, Gertz MA. Primary systemic amyloidosis: Clinical and laboratory features in 474 cases. Semin J Hematol 1995;32:45–59.
10. Hausfater P, Costedoat-Chalumeau N, Amoura Z, et al. AL cardiac amyloidosis and arterial thromboembolism. Scand J Rheumatol 2005; 34:315–9.
11. Roberts WC, Waller BF. Cardiac amyloidosis causing cardiac dysfunction: analysis of 54 necropsy patients. Am J Cardiol 1983;52:137–46.
12. Falk RH, Alexander KM, Liao R, Dorbala S. AL (Light- Chain) Cardiac Amyloidosis: A Review of Diagnosis and Therapy. Am J Cardiol 2016;68:1323–41.
13. Stables RH, Ormerod OJ. Atrial thrombi occurring during sinus rhythm in cardiac amyloidosis: Evidence for atrial electromechanical dissociation. Br J Card Soc 1996;75:426.
14. Feng D, Syed IS, Martinez M, et al. Intracardiac thrombosis and anticoagulation therapy in cardiac amyloidosis AHA J Hematol 2009;119:2490-7.
15. Dispenzieri A, Gertz MA, Buadi F. What do I need to know about immunoglobulin light chain (AL) amyloidosis? Blood Review 2012;26:137–54.