“THE LIVER IN AMYLOIDOSIS: AN ANALYSES OF THE INSTITUTIONAL AMYLOIDOSIS REGISTRY

ADELA AGUIRRE; MARIA LOURDES POSADAS MARTINEZ; MARCIANO SEBASTIAN

Congreso; XXVII Congreso ALEH,; 2022

En representación del Comité Científico de la Asociación Latinoamericana para el Estudio del Hígado ALEH, tenemos el agrado de comunicarle que el trabajo titulado: “THE LIVER IN AMYLOIDOSIS: AN ANALYSES OF THE INSTITUTIONAL AMYLOIDOSIS REGISTRY” ha sido seleccionado para ser presentado en modalidad E-POSTER (P-29), en el XXVII Congreso ALEH, que se realizará del 6 al 9 de septiembre del presente año, en Buenos Aires, Argentina.Título: THE LIVER IN AMYLOIDOSIS: AN ANALYSES OF THE INSTITUTIONAL AMYLOIDOSIS REGISTRYAutores: MARIA ADELA AGUIRRE1 , MARCELINA CARRETERO2, EUGENIA VILLANUEVA3, ELSA MERCEDES NUCIFORA4, MARÍA SOLEDAD SAEZ5, ERIKA BÁRBARA BRULC4, DIEGO PEREZ DE ARENAZA3, SEBASTIÁN MARCIANO6, MARÍA AGUSTINA MARCO1, GISELA BENDELMAN1, PATRICIA BEATRIZ SORROCHE5, MARIA LOURDES POSADAS MARTÍNEZ1,5.Lugar de trabajo: 1 Servicio de Clínica Médica, Hospital Italiano de Buenos Aires, Argentina. Instituto de Medicina Traslacional e Ingeniería Biomédica (IMTIB) Unidad Ejecutora del CONICET.2 Área de Investigación en Medicina Interna, Servicio de Clínica Médica, Hospital Italiano de Buenos Aires. 3 Servicio de Cardiología, Hospital Italiano de Buenos Aires, Argentina. 4 Servicio de Hematología, Hospital Italiano de Buenos Aires, Argentina.5 Instituto de Medicina Traslacional e Ingeniería Biomédica (IMTIB) Unidad Ejecutora del CONICET. Área de Investigación No Patrocinada, Departamento de Investigación, Hospital Italiano de Buenos Aires. Área de Investigación en Medicina Interna, Servicio de Clínica Médica, Hospital Italiano de Buenos Aires. 6 Sección de Hepatología, Hospital Italiano de Buenos Aires, Argentina.INTRODUCTION: The liver can be either compromised by infiltrative damage of amyloid, as it happens in AL and AA amyloidosis or be its cause, as it occurs in transthyretin TTR-related amyloidosis. In the latter, the liver synthesizes a defective variant TTR which has the capacity for cardiac, neurological, and renal damage, but liver function is preserved. OBJECTIVE: To describe the clinical characteristics and prognosis of patients with liver involvement of amyloidosis (AL and AA) METHODS: Retrospective cohort of patients with hepatic involvement included in the Institutional Amyloidosis Registry (ClinicalTrials.gov NCT01347047) between June 2010 and January 2022. Clinical characteristics and complementary studies were analyzed, as well as their evolution. RESULTS: 359 patients with amyloidosis were included in the registry, of whom 16 (5% (CI 2.7-7.3)) had liver involvement. The most frequent types of amyloidosis were: AL 88% (14), AA 6% (1) and non-typed 6% (1). The median age at diagnosis was 64 years (IR 63-74), male 44% (7). The median albumin value was 3.0 gr/dL (IR 2.5-3.8), alkaline phosphatase 705 IU (IR 395-114), total bilirubin mg/dL 1.1 (IR 0.5-14.8), and more than 25% had jaundice. Thirty-one percent presented cardiac compromise. The mortality rate in the study period was 56% (CI 30%-80%). When comparing patients with amyloidosis with and without liver involvement, mortality was higher in the liver involvement group (29% vs 56%, p 0.02).CONCLUSION: ​​We present the first report in our region with adequate sampling that allows us to approximate the burden of this disease in relation to the liver. Hepatic infiltrative involvement has a high mortality rate in amyloidosis ​​compared to those without liver involvement.