AL amyloidosis in Argentina: the Hospital Italiano de Buenos Aires

ADELA AGUIRRE; MARIA LOURDES POSADAS MARTINEZ; ET AL

Congreso; The XVIth International Symposium on Amyloidosis: ISA2018; 2018

Elsa Mercedes Nucifora, María Adela Aguirre,María Lourdes Posadas Martínez, Patricia Sorroche, María Soledad Saez, Dorotea Fantl, Bruno Rafael Boietti, Julieta Antonella Rocca, Diego Perez de Arenaza, Carlos Federico Varela, Gustavo Greloni , Hernán García Rivello, Ana Lisa Basquiera, Jorge Alberto Alberbide, Diego Hernán Giunta. Department of Medicine, Hospital Italiano de Buenos Aires, Grupo de Estudio de la Amiloidosis, Buenos Aires, Argentinaelsa.nucifora@hospitalitaliano.org.arBACKGROUND: The Hospital Italiano de Buenos Aires is a University Hospital, that receives patients from across the country and neighboring countries for diagnosis and treatment. The purpose of this presentation is to show our experience with AL Amyloidosis.METHOD: We included all AL patients of the Institutional Amyloidosis Registry of the Hospital Italiano de Buenos Aires (IAR), in an observational study, from 04/2012 to 05/2017. RESULTS: Out of the 186 patients of the IAR, 72 are AL (39%). Female 35 (49%), with a median age of 61 (SD 13) range 35 - 89 years. 26 patients (36%) had a monoclonal spike on the proteinogram, and 6 of them (8%) had a concentration higher than 1.5 g/dL.The isotype distribution of the monoclonal components was: L-lambda 44%, IgG-lambda 28%, IgG-kappa 10%, L-kappa 6% and other isotypes 12%. Kappa/lambda ratio 1/3.7 mg/L (in MM 2/1). Bone marrow plasmocytes ranged from 0 to 40%. Organ compromise at diagnosis were: heart 53 patients (74%), Kidney: 36 patients (50%), soft tissues 15 patients (20%) digestive tract 12 patients (17%) (no systematic search), hepatomegaly 4 patients (5%) polyneuropathy 4 (5%). Eight patients died before being able to initiate some type of treatment, mainly by heart failure. The rest were followed and are the reason for the presentation. Fifteen patients were lost of follow up. The majority received treatment with Bortezomib, between 4 and 13 cycles (mean 6). Six patients died at the start of treatment (grade III heart failure). Another six patients needed a second line treatment. Eleven patients met the criteria for autotransplant (three with previous heart transplant). Ten live, mean follow-up 41 months (2-123 months). The overall survival at one year was 69.2% (95% CI 51.2-82) and at three years 52.7% (95% CI 34.4-68). Patients with cardiac compromise had a 50% mortality of at 2 years. Patients with renal compromise had a similar survival to the general population, when they entered on hemodialysis they could not leave it. Two patients in complete remission are on renal transplant list. 14 patients had a autotransplantation.CONCLUSION: Early diagnosis,prevents organic failure and is a major determinant for survival. Late diagnosis requires rescue treatments such as heart transplantation with high mortality. Cardiac involvement is the most important prognostic factor, especially if the patient arrives in clinical heart failure. Treatment with chemotherapy and autotransplantation offers the best possibilities for survival in an early diagnosis setting.