AL amyloidosis in Argentina: Hospital Italiano de Buenos Aires

ELSA MERCEDES NUCIFORA; MARÍA ADELA AGUIRRE ; PATRICIA SORROCHE ; MARÍA SOLEDAD SAEZ ; DOROTEA FANTL; JULIETA ANTONELLA ROCCA; DIEGO PEREZ DE ARENAZA; CARLOS FEDERICO VARELA; GUSTAVO GRELONI; HERNÁN GARCÍA RIVELLO; ANA LISA BASQUIERA; JORGE ALBERTO ALBERBIDE; DIEGO HERNÁN GIUNTA; BRUNO RAFAEL BOIETTI; MARIA LOURDES POSADAS MARTINEZ

AMYLOID

INFORMA HEALTHCARE

Año: 2019

1350-6129

Title: AL amyloidosis in Argentina: Hospital Italiano de Buenos AiresAuthors: Elsa Mercedes Nucifora1, María Adela Aguirre1, Patricia Sorroche2, María Soledad Saez2, Dorotea Fantl1, Julieta Antonella Rocca1, Diego Perez de Arenaza1, Carlos Federico Varela3, Gustavo Greloni3, Hernán García Rivello4, Ana Lisa Basquiera1, Jorge Alberto Alberbide1, Diego Hernán Giunta1, Bruno Rafael Boietti1, and María Lourdes Posadas Martínez1. 1Department of Medicine, 2 Department of Laboratory, 3 Nephrology service, 4 Pathological anatomy service, Hospital Italiano de Buenos Aires (HIBA), Grupo de Estudio de la Amiloidosis, Buenos Aires, ArgentinaCorrespondence: Elsa Nucifora, Department of Internal Medicine, Hematology. HIBA, Perón 4190, Buenos Aires Argentina. E-mail: elsa.nucifora@hospitalitaliano.org.arBackgroundAl amyloidosis incidence rate adjusted to Buenos Aires is 6 (95%CI: 2.6-9.7) per 1,000,000 person-years [1]. Prognosis depends on the organ dysfunction [2] and in the extent of cardiac involvement. Although systemic AL amyloidosis is not cured, it improves with early diagnosis and therapy [3]. Treatment options depend on extension and localization of disease and comorbidities. Patients are eligible to autologous hematopoietic cell transplantation with good performance status, less comorbidities and less organ dysfunction. No eligible patients are given chemotherapy, bortezomib-based triplet [4][5]. Material and MethodsProspective cohort of consecutive adults with positive amyloid staining by Congo red in any tissue of the Institutional Amyloidosis Registry of the Hospital Italiano de Buenos Aires (IAR) between April 2010 to May 2017. For this study only AL amyloidosis is included, diagnoses is based on presence of an amyloid-related systemic syndrome, and evidence of a monoclonal plasma cell proliferative disorder (serum or urine M protein, abnormal serum free light chain ratio, or clonal plasma cells in the bone marrow). Follow-up was performed through medical electronic records and/or structured phone call interviews. Survival was calculated with the Kaplan?Meier Analyses with stata version 11ResultsDuring the period of 186 patients were included in the IAR, of those 39% (72) were diagnosis of AL amyloidosis. Female 49% (35), with a median age of 61 years (IR 35 - 89). Thirty six patients (26) had a monoclonal spike on the proteinogram, and 8% of them (6) had a concentration higher than 1.5 g/dL. The most frequent 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%. The most frequent organ compromise at diagnosis were: heart 74% (53), kidney 50% (36), soft tissues 20%(15), gastrointestinal tract 17% (12), hepatomegaly 5% (4) and nerve (polyneuropathy) 5% (4). Eight patients died before being able to initiate some type of treatment, mainly by heart failure. 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). Additionally, six patients needed a second line treatment. Eleven patients met the criteria for autotransplant (three with previous heart transplant) with mean follow-up 41 months (IR 2-123 months), only one patient died. Two patients in complete remission are on renal transplant list. The overall survival at one year was 69% (CI 51-82) and at three years 53% (CI 34-68). Mortality has higher in patients with cardiac compromise, with a rate of 50% 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. ConclusionEarly 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.The authors report no conflicts of interest.