Amiloidosis primaria asociada a mielofibrosis idiopática crónica. Una relación inusual

Introduction: amyloidosis is the term used for diseases caused by the extracellular deposition of protein fibrils in tissues and organs. These diseases are defined and classified by the biochemical nature of the protein in the fibril deposits. Clinical case: it is presented a clinical case of a pati...

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Detalles Bibliográficos
Autores: Pila Pérez, Rafael, Rosales Torres, Pedro, Pila Peláez, Rafael, Holguín Prieto, Víctor A., Ramírez Lana, Lázaro
Tipo de recurso: artículo
Estado:Versión publicada
Fecha de publicación:2012
País:Colombia
Institución:Universidad Industrial de Santander
Repositorio:Repositorio UIS
Idioma:español
OAI Identifier:oai:noesis.uis.edu.co:20.500.14071/5689
Acceso en línea:https://revistas.uis.edu.co/index.php/revistamedicasuis/article/view/3152
https://noesis.uis.edu.co/handle/20.500.14071/5689
Access Level:acceso abierto
Palabra clave:Primary Amyloidosis
Myelofibrosis
Extramedullary Hematopoiesis
Amiloidosis
Mielofibrosis
Hematopoyesis Extramedular
Descripción
Sumario:Introduction: amyloidosis is the term used for diseases caused by the extracellular deposition of protein fibrils in tissues and organs. These diseases are defined and classified by the biochemical nature of the protein in the fibril deposits. Clinical case: it is presented a clinical case of a patient with the diagnosis of primary amyloidosis and chronic idiopathic myelofibrosis with extramedullary hematopoiesis in the spleen. A 61-year male patient is admitted due to presenting infiltrative restrictive cardiomyopathy, hematologic alterations, hepatosplenomegalia and sensorimotor neuropathy. Amyloidosis is diagnosed through bone marrow biopsy, while fine-needle aspiration cytology of the spleen demonstrates extramedullary hematopoiesis, and along with hematologic studies, it is concluded as a chronicidiopathic myelofibrosis. Conclusions: in Universitary Hospital, imaging and histological studies are important to confirm the diagnosis of amyloidosis. The causality between primary amyloidosis and chronic idiopathic myelofibrosis there has not been reported in the literature. (MÉD.UIS. 2012;25(2):137-44)