A case report of al amyloidosis
Article Sidebar
Main Article Content
Abstract
Background: amyloidosis is a systemic disease resulting from the deposition of misfolding proteins, in immunoglobulin light chain amyloidosis (AL) fibrils are composed of fragments of monoclonal light chains. In Argentina the incidence density of AL amyloidosis is 4.54 per million people year. Case: a 71-year-old female patient who consults for neural pain located in the left upper limb, associated with edemas in both lower limbs and exertional dyspnea, weight loss, constipation and macroglossia. On physical examination she had blood pressure of 100/60 mmHg, afebrile, saturating 98% of ambient air, weight of 46 kg and body mass index of 18.9. The peripheral blood laboratory has Kappa free light chains: 5.8 mg/L, Lambda: 430 mg/L with K L ratio: 0.13 mg/L and a ProBNP of 1686 pg/mL. Abdominal fat biopsy reports positive Congo red staining. Cardiac magnetic resonance with contrast (gadolinium) has been performed and result compatible with cardiac amyloidosis. Chest CT showed an area of consolidation in the upper lobe of the right lung; positive congo red. With the diagnosis of AL amyloidosis she was treated with CYBORD. Autologous stem cell transplantation was performed. Discussion: cardiac involvement is the main cause of morbidity and mortality by amyloidosis
Downloads
Article Details
Section
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
How to Cite
References
Sher T, Gertz MA. Stem cell transplantation for immunoglobulin light chain amyloidosis. Curr Probl Cancer. 2017; 41(2):129-37. DOI: https://doi.org/10.1016/j.currproblcancer.2017.03.001
Grogan M, Dispenzieri A, Gertz MA. Light-chain cardiac amyloidosis: strategies to promote early diagnosis and cardiac response. Heart. 2017; 103(14):1065-72. DOI: https://doi.org/10.1136/heartjnl-2016-310704
Vázquez J, Durante A, Hernández F, et al. Should we amplify cardiac response criteria in patients with cardiac light-chain amyloidosis treated with Bortezomib? J Heart Lung Transplant. 2017;36:S169. DOI: https://doi.org/10.1016/j.healun.2017.01.444
Lee GK, Feng D, Grogan M, et al. Cardiac amyloidosis: typing, diagnosis, prognosis and management. En: Feng D, ed. Amyloidosis [Internet]. [Rijeka]: InTech; 2013 June 12 [citado 05/09/2017]. DOI: 10.5772/53763. Disponible en: https://www.intechopen.com/books/amyloidosis/cardiac-amyloidosis-typing-diagnosis-prognosis-and-management
Wechalekar AD, Gillmore JD, Hawkins PN. Systemic amyloidosis. Lancet. 2016; 387(10038):2641-54. DOI: https://doi.org/10.1016/S0140-6736(15)01274-X
Rapezzi C, Merlini G, Quarta CC, et al. Systemic cardiac amyloidoses: disease profiles and clinical courses of the 3 main types. Circulation. 2009; 120(13):1203-12. DOI: https://doi.org/10.1161/CIRCULATIONAHA.108.843334