Resumo

PILA PEREZ, Rafael et al. Primary amyloidosis associated to chronic idiopathic myelofibrosis. An unusual relationship. Medicas UIS [online]. 2012, vol.25, n.2, pp.137-144. ISSN 0121-0319.

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 chronic idiopathic 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)

Palavras-chave : Primary Amyloidosis; Myelofibrosis; Extramedullary Hematopoiesis.

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