Hepatoesplenomegalia Secundario a Síndrome Antifosfolipídico

Abstract

Also called antiphospholipid syndrome antiphospholipid syndrome, anticardiolipin syndrome, antiphospholipid syndrome cofactor, Hughes syndrome; and in France known as Soulier-Boffe syndrome it is an autoimmune hypercoagulable state caused by antibodies directed against phospholipids of cell membranes, causing increased susceptibility to the formation of intravascular clots (thrombosis) in both arteries and veins, also complications related to pregnancy such as spontaneous abortions, fetal death, preterm delivery, or severe preeclampsia. The etiology and pathogenesis of these antibodies are not elucidated, the wide range of clinical manifestations could be explained by multiple mechanisms, it is said that this syndrome occurs due to an autoimmune disorder that leads to the production of autoantibodies directed against components of cell membranes called phospholipids (antiphospholipid antibodies or aPL). Thrombosis is a major disease mechanisms, primarily caused by the activation of endothelial cells, monocytes and platelets. Now it considered as one of the most frequently acquired and cerebrovascular accident (CVA) in under 50 causes of thrombophilia. More than 50% of lupus patients with aPL eventually develop SAF. The term "primary antiphospholipid syndrome" is used when the SAF occurs in the absence of other autoimmune diseases, while the term "secondary antiphospholipid syndrome" is used when the SAF occurs in the context of other autoimmune diseases such as systemic lupus erythematosus (SLE). In some rare cases, an EFF may lead to multisystem failure (multiple organ failure), sudden due to widespread thrombosis; in this case usually use the term catastrophic antiphospholipid syndrome, and presents a high risk of death. The evaluation is carried out mainly in a thorough medical history, physical examination and laboratory tests for antibodies phospholipid cardiolipin antibodies (aCL), anti beta2 glycoprotein I antibodies, lupus anticoagulant. For the diagnosis of antiphospholipid syndrome should be present at least one clinical and one laboratory finding. The goal of prophylactic treatment is to keep the patient INR between 2.0 and 3.0, however it is not often done with patients without any kind of thrombotic symptoms; During pregnancy, they heparins and low molecular weight low-dose aspirin instead of warfarin are used, due to the teratogenicity of warfarin. Women who have experienced recurrent spontaneous abortions are given prophylactic treatment with aspirin and initiate treatment with low molecular weight heparin disappears just recognize that their menstrual cycle. In refractory cases it can be used plasmapheresis.