Amiloidosis AL: conceptos actuales

Palabras clave: amiloidosis AL, cadenas ligeras inmunoglobulina, mutaciones, proteólisis, plegamiento, proteínas.

Resumen

Las amiloidosis sistémicas constituyen un grupo de enfermedades con diversas etiologías, caracterizadas por la síntesis de proteínas con plegado defectuoso, capaces de agregarse y depositarse en el medio extracelular de diferentes órganos y tejidos, alterando su estructura y función. Se conocen más de 14 formas de amiloidosis sistémica, de las cuales la más frecuente es la amiloidosis AL, objeto de esta revisión, en la que las proteínas precursoras son cadenas ligeras de inmunoglobulina inestables, secretadas por un clon de células plasmáticas o, con menor frecuencia, por un linfoma linfoplasmocítico o de células del manto. La amiloidosis AL puede llevar a una amplia gama de manifestaciones clínicas y compromiso de órganos, como el corazón y el riñón. El reconocimiento temprano de la enfermedad y el diagnóstico oportuno son determinantes para mejorar la supervivencia de los pacientes. El tratamiento deberá ser individualizado de acuerdo con la condición de cada paciente, lo que hace necesaria una correcta clasificación de los individuos según su pronóstico. La terapia dirigida a la amiloidosis está enfocada esencialmente en disminuir el compromiso orgánico, y por ende, prolongar la supervivencia con mejoría en los síntomas. En esta revisión se discutirán aspectos importantes de la fisiopatología, epidemiología, manifestaciones clínicas, diagnóstico
y tratamiento de la amiloidosis AL.

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Biografía del autor/a

Jorge Andrés Lacouture-Fierro, Universidad de Antioquia

Médico, Especialista en Medicina Interna, Fellow de Hematología Clínica, Universidad de Antioquia. Medellín, Colombia. https://orcid.org/0000-0002-8398-5807

Leonardo Mejía-Buriticá, Hospital Universitario San Vicente Fundación, IPS Universitaria

Médico, Especialista en Medicina Interna, Especialista en Hematología, Hospital Universitario San Vicente Fundación, IPS Universitaria. Medellín, Colombia. https://orcid.org/0000-0003-4570-2762

Daniel Andrés Ribero-Vargas, Universidad de Antioquia

Médico, Residente de Medicina Interna, Universidad de Antioquia. Medellín, Colombia. https://orcid.org/0000-0002-5402-4599

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Cómo citar
1.
Lacouture-Fierro JA, Mejía-Buriticá L, Ribero-Vargas DA. Amiloidosis AL: conceptos actuales. Med. Lab. [Internet]. 4 de abril de 2022 [citado 5 de octubre de 2022];26(2):119-3. Disponible en: https://medicinaylaboratorio.com/index.php/myl/article/view/572
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2022-04-04
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