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- W1488792092 abstract "The antiphospholipid syndrome (APS) is an acquired prothrombotic syndrome characterized by venous or arterial thromboses and pregnancy morbidity. It can present as primary APS without any discernable underlying disease, or in association with systemic autoimmune disease [usually systemic lupus erythematosus (SLE)], infections (mainly chronic viral infections) and malignant process, among others. It may also occur rapidly over days or weeks, when it is known as catastrophic APS (CAPS).The first study described one of the largest known cohorts of patients with primary APS from 4 different referral centers. The final study sample included 128 patients with primary APS with a median age of 42 years and mean follow-up of 9 years. After a median disease duration of 8.2 years, 110 (86%) patients remained with primary APS; 11 (8%) patients developed SLE; 6 (5%), LLD; and 1 (1%), myasthenia gravis. At the end of the study, 113 (88%) patients were alive and 15 (12%) patients had died. Our study confirms that progression from primary APS to SLE or LLD is unusual, even after a long follow-up.In the second study, we evaluated 120 cases of antiphospholipid antibodies associated with malignancies with a mean age of 56 years, The main hematological malignancies found were B-cell lymphoma, spleen lymphoma and chronic myeloid leukemia. The main solid tumors were renal cell carcinoma, primary tumor of unknown origin, lung adenocarcinoma and breast carcinoma. Around one third of patients achieved aPL remission after treatment.In the third study, we analyzed 15 cases of CAPS that appeared during pregnancy or the puerperium with a mean age at the time of the CAPS event of 27 years. In 7 of the 14 (50%) cases, CAPS appeared during pregnancy, in 6 (43%) cases it presented during puerperium and in 1 (7%) after curettage for a fetal death. The main clinical and serological characteristics were similar to those of patients with CAPS triggered by other factors, however we found some particular features including placental infarctions, pelvic vein thrombosis and myometrial thrombotic microangiopathy and HELLP syndrome.Final conclusion: Primary APS is a widely recognized distinct entity which rarely progresses to SLE, even after long-term follow-up. APS may also be associated with other chronic disorders, such as solid tumors or hematological malignancies. In cases with the life-threatening variant of APS known as CAPS, pregnancy and the puerperium are periods of high susceptibility for the development of this often fatal form of presentation.RESUMEN:SINDROME ANTIFOSFOLIPIDICO: EXPANDIENDO EL ESPECTRO CLINICA DE LA TROMBOSIS AUTOINMUNEEl sindrome antifosfolipidico (SAF) es un sindrome protrombotico adquirido caracterizado por trombosis venosas y arteriales y perdidas fetales recurrentes. Puede estar presente como SAF primario cuando no esta asociado a ninguna enfermedad autoinmune [fundamentalmente el lupus eritematoso sistemico (LES)] o en asociacion a otros procesos tales como infecciones y procesos neoplasicos, entre otros. Tambien puede manifestarse de una forma acelerada en dias o semanas, caracterizado por trombosis de pequenos organos y fallo multiorganico, lo que se conoce como SAF catastrofico.En el primer estudio se analizo una de las series mas amplia y con mas largo seguimiento de pacientes con SAF primario. Se incluyeron 128 pacientes con un seguimiento medio de 9 anos. Despues de una duracion media de la enfermedad de 8 anos, 110 (86%) pacientes continuan con el diagnostico de SAF primario, 11 (8%) pacientes desarrollaron un LES, 6 (5%) una forma incompleta de lupus (lupus-like disease) y 1 (1%) paciente desarrollo una miastenia gravis. La presencia del test de Coombs positivo confiere un riesgo estadisticamente significativo para el desarrollo de LES. . Nuestro estudio confirma que es inusual que un SAF primario evolucione hacia un LES o una forma incompleta de lupus, incluso tras un periodo largo de seguimiento.En el segundo estudio se incluyeron un total de 120 casos con anticuerpos antifosfolipidicos (AAF) asociados a procesos neoplasicos. Las principales neoplasias hematologicas relacionadas a los AAF fueron el linfoma de celulas B, el linfoma esplenico y la leucemia mieloide cronica. Los principales tumores solidos fueron el carcinoma de celulas renales, los tumores de primario desconocido, el adenocarcinoma de pulmon y el cancer de mama. Alrededor de una tercera parte de los paciente negativizaron los AAF despues del tratamiento de la neoplasia.En el tercer estudio se analizaron 15 pacientes con SAF catastrofico que ocurrieron durante el embarazo o el puerperio. Las caracteristicas clinicas generales del SAF catastrofico durante el embarazo o el puerperio fueron similares a las del SAF catastrofico desencadenado por otros factores a excepcion de una tasa mayor de abortos previos. Sin embargo se encontraron una serie de caracteristicas particulares, como el sindrome de HELLP, la trombosis placentaria, la microangiopatia trombotica de miometrio o la trombosis de la vena pelvica.CONCLUSION FINAL: El SAF primario es una entidad propia ampliamente reconocida que en raras ocasiones evoluciona a un LES, incluso tras un periodo largo de seguimiento. El SAF puede asociarse a una serie de procesos cronicos como lo son las neoplasias hematologicas y los tumores solidos. En aquellos casos con la variante catastrofica del SAF, el embarazo y el puerperio, constituyen un periodo de alta susceptibilidad para el desarrollo de esta variante altamente letal del SAF. " @default.
- W1488792092 created "2016-06-24" @default.
- W1488792092 creator A5018350354 @default.
- W1488792092 creator A5060384807 @default.
- W1488792092 date "2007-10-03" @default.
- W1488792092 modified "2023-09-24" @default.
- W1488792092 title "Antiphospholipid Syndrome: Expanding the Spectrum of Autoimmune Thrombosis" @default.
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