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      Ruptura esplénica espontánea secundaria a amiloidosis

      case-report

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          Abstract

          La ruptura esplénica se produce frecuentemente por mecanismos traumáticos. Se la denomina espontánea o patológica cuando no existe tal antecedente o hay alguna patología esplénica predisponente. La ruptura esplénica espontánea por infiltración amiloide es una causa muy infrecuente de abdomen agudo quirúrgico. Se presenta el caso de una mujer internada por abdomen agudo secundario a ruptura esplénica espontánea, cuyo diagnóstico final fue amiloidosis primaria.

          Translated abstract

          Splenic rupture is frequently produced due to traumatic mechanisms, being referred to as spontaneous or pathological when there is no history of trauma or there is an underlying splenic disease. Spontaneous splenic rupture due to amyloid deposits is a very uncommon cause of surgical acute abdomen. The patient in this case is a woman admitted with acute abdomen secondary to spontaneous splenic rupture, whose final diagnosis was primary amyloidosis.

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          Most cited references9

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          Monoclonal gammopathy of undetermined significance.

          Significant advances have been made in our understanding of the natural history, pathogenesis, mechanisms of progression and prognosis of monoclonal gammopathy of undetermined significance (MGUS). Although the overall incidence of MGUS progression is 1 per year, it is now possible to more accurately predict the risk of progression based on a new risk-stratification model. However, it is still hard to design chemopreventive trials given that the absolute risk of progression per year is low, even in the high-risk group. Therefore, further improvements in estimating the risk of progression are needed. Roughly 50% of MGUS may originate from primary translocation events at the heavy-chain immunoglobulin locus at chromosome 14q32. In most of the remaining MGUS patients, the initiating event is associated with genomic instability that results in hyperdiploidy of certain odd numbered chromosomes. Cytogenetically distinct subtypes of MGUS may carry significant differences in the risk of progression to malignancy. New markers, such as measures of bone marrow angiogenesis and circulating plasma cells may be additional prognostic factors. A better understanding of the mechanisms underlying the transition of normal plasma cells to the MGUS phenotype, and the transition of MGUS to myeloma or related malignancy, will help identify new risk factors for progression and new targets for chemopreventive interventions.
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            Spontaneous splenic rupture in two patients with a blastoid variant of mantle cell lymphoma.

            Spontaneous rupture of the spleen is a rare complication of hematological malignancies, occurring most commonly in patients with acute leukemia, but it has been documented in chronic leukemias and also in lymphomas. We report two patients with histologically and immunohistochemically confirmed mantle cell lymphoma (MCL) who experienced a spontaneous splenic rupture. An 80-year-old woman and a 51-year-old man had a blastoid variant of MCL and responded poorly to conventional treatment. Both patients recovered after splenectomy. The woman died of progressive lymphoma 2 months later. An allogeneic bone marrow transplantation was performed in the man with a good initial result, but an aggressive relapse was seen only 6 months later and he died of progressive lymphoma. In view of our data, we suggest special caution when MCL is complicated by rapid progression and severe splenomegaly. Although it is a rare phenomenon, the risk of splenic rupture should be kept in mind.
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              Spontaneous splenic rupture in two patients with a blastoid variant of mantle cell lymphoma

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                Author and article information

                Contributors
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Journal
                medba
                Medicina (Buenos Aires)
                Medicina (B. Aires)
                Fundación Revista Medicina (Ciudad Autónoma de Buenos Aires )
                1669-9106
                April 2005
                : 65
                : 2
                : 151-153
                Affiliations
                [1 ] Hospital Alemán Argentina
                Article
                S0025-76802005000200012
                70f7b06c-a91a-41d6-8bd7-a2d87489da81

                http://creativecommons.org/licenses/by/4.0/

                History
                Product

                SciELO Argentina

                Self URI (journal page): http://www.scielo.org.ar/scielo.php?script=sci_serial&pid=0025-7680&lng=en
                Categories
                MEDICINE, GENERAL & INTERNAL

                Internal medicine
                ruptura esplénica,amiloidosis,abdomen agudo,splenic rupture,amyloidosis,acute abdomen

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