ABSTRACT
Las amiloidosis son un grupo heterogéneo de enfermedades caracterizadas por el depósito extracelular enlos órganos y tejidos de una serie de proteínas fibrilares, no relacionadas bioquímicamente entre sí, pero que comparten unas características comunes que incluyen la birrefringencia verde manzana con la luz polarizada, previa tinción con rojo Congo, y la configuración en hoja plegada beta-laminar por difracción de rayos X. El depósito de amiloide puede formarse en muchos órganos (amiloidosis sistémicas), o puede afectara un único tejido (amiloidosis localizadas u organolimitadas).Existen numerosas clasificaciones, pero en esta revisión las amiloidosis están ordenadas según el cuadro clínico, que está determinado según la proteína del amiloide implicada. Se presta especial atención a las manifestaciones cutáneo mucosas que con frecuencia son el primer signo de la enfermedad, que son útiles para el diagnóstico precoz y evitan así exploraciones más agresivas. También se analiza la afectación de otros órganos, el diagnóstico, pronóstico y tratamiento delas amiloidosis sistémicas
Amyloidoses are a heterogeneous group of diseases characterized by extracellular fibrillar protein deposits inthe organs and tissues. These proteins are not biochemically related to each other, but share certain common characteristics, including apple green birefringence with polarized light after staining with Congo red, and beta-pleated sheet configuration through x-ray diffraction. Amyloid deposits may occurin many organs (systemic amyloidoses) or may affect a singletissue (localized or organ-specific amyloidoses). There are different classifications, but in this review the amyloidoses are organized by clinical symptoms, which are determined by theamyloid protein involved. Special attention is given to cutaneous and mucous membrane manifestations, which are often the first sign of the disease and are useful for early diagnosis, thus avoiding more aggressive procedures. The involvement of other organs is analyzed, as are the diagnosis, prognosis and treatment of systemic amyloidoses
Subject(s)
Male , Female , Humans , Amyloidosis/classification , Amyloidosis/diagnosis , Amyloidosis/therapy , Amyloid/analysis , Amyloid/classification , Heart Failure/complications , Proto-Oncogene Proteins c-mos/pharmacology , Proto-Oncogene Proteins c-mos/therapeutic use , Amyloidosis/epidemiology , Amyloidosis/pathology , Paraproteinemias/complications , Paraproteinemias/diagnosis , Paraproteinemias/pathology , Gastrointestinal Hemorrhage/complications , Gastrointestinal Hemorrhage/diagnosis , Amyloidosis, Familial/complications , Amyloidosis, Familial/diagnosis , Amyloidosis, Familial/pathologyABSTRACT
Amyloidoses are a heterogeneous group of diseases characterized by extracellular fibrillar protein deposits in the organs and tissues. These proteins are not biochemically related to each other, but share certain common characteristics, including apple green birefringence with polarized light after staining with Congo red, and beta-pleated sheet configuration through x-ray diffraction. Amyloid deposits may occur in many organs (systemic amyloidoses) or may affect a single tissue (localized or organ-specific amyloidoses). There are different classifications, but in this review the amyloidoses are organized by clinical symptoms, which are determined by the amyloid protein involved. Special attention is given to cutaneous and mucous membrane manifestations, which are often the first sign of the disease and are useful for early diagnosis, thus avoiding more aggressive procedures. The involvement of other organs is analyzed, as are the diagnosis, prognosis and treatment of systemic amyloidoses.
