Inhibidores adquiridos de la coagulación en una paciente con lupus eritematoso sistémico y anticuerpos antifosfolipídicos: respuesta a rituximab / Aquired inhibitors of coagulation in a patient with systemic lupus erythematosus and antiphospholipid antibodies: response to rituximab

Las manifestaciones hematológicas de los pacientes con lupus eritematoso sistémico (LES) son diversas. Aunque el desarrollo de anticuerpos antifosfolipídicos se asocia con una prolongación in vitro de los tiempos de coagulación, las manifestaciones clínicas suelen ser trombóticas. Los anticoagulantes circulantes que aparecen en pacientes sin anomalías previas de la coagulación son consecuencia del desarrollo de autoanticuerpos contra los factores II, V, VII, VIII y IX, XI, XIII, factor de Von Willebrand u otras glucoproteínas de membrana. Todos ellos condicionan coagulopatías poco comunes, 1 caso por millón de personas por año, y que pueden comprometer seriamente la vida de los pacientes. Presentamos el caso de una mujer con LES y anticuerpos antifosfolipídicos, que presentó síndrome de Evans, como primera manifestación, y posteriormente desarrolló un síndrome hemorrágico, presumiblemente por anticuerpos contra múltiples factores de la cascada de coagulación, sin respuesta a dosis altas de esteroides e inmunosupresores, que finalmente respondió a rituximab (AU)

Hematologic signs of systemic lupus erythematosus are diverse (SLE). Although a delayed coagulation time is anti-phospholipid antibody related, thrombotic events are the usual clinical manifestation. Spontaneous appearance of circulating anticoagulant in the absence of a previous coagulation disorder is secondary to the development of antibodies to factors II, V, VIII, IX, XI, XII, vonWillebrand, and other membrane glucoproteins, all of them uncommon causes (1 case per million persons per year) of life threatening coagulopathies. We report a case of SLE and antiphospholipid antibodies in a woman with a hemorrhagic syndrome, probably caused by multiple antibodies to coagulation factors, unresponsive to steroids and high-dose immunosupressive therapy and a favorable response to rituximab (AU)

[Aquired inhibitors of coagulation in a patient with systemic lupus erythematosus and antiphospholipid antibodies: response to rituximab]. / Inhibidores adquiridos de la coagulación en una paciente con lupus eritematoso sistémico y anticuerpos antifosfolipídicos: respuesta a rituximab.

Hematologic signs of systemic lupus erythematosus are diverse (SLE). Although a delayed coagulation time is anti-phospholipid antibody related, thrombotic events are the usual clinical manifestation. Spontaneous appearance of circulating anticoagulant in the absence of a previous coagulation disorder is secondary to the development of antibodies to factors II, V, VIII, IX, XI, XII,vonWillebrand, and other membrane glucoproteins, all of them uncommon causes (1 case per million persons per year) of life threatening coagulopathies. We report a case of SLE and antiphospholipid antibodies in a woman with a hemorrhagic syndrome, probably caused by multiple antibodies to coagulation factors, unresponsive to steroids and high-dose immunosupressive therapy and a favorable response to rituximab.