[Thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus. Clinical diagnosis and therapeutic strategies]. / Porpora trombotica trombocitopenica associata a lupus eritematoso sistemico. Inquadramento clinico-diagnostico e strategie terapeutiche.

The aim of our work is the focusing on some aspects about both the etiopathogenesis of thrombotic thrombocytopenic purpura and the therapeutic choices required to strongly reduce the mortality. Moreover the article reviews the association between thrombotic thrombocytopenic purpura and systemic lupus erythematosus. Thrombotic thrombocytopenic purpura is a rare and severe hematologic syndrome, first described in 1924, characterized by a clinical pentade: fever, microangiopathic anemia, thrombocytopenia, neurologic abnormalities and renal involvement. It is unknown if the endothelial damage represents the first lesion or if the platelet hyperaggregability precedes the vascular injury. Some data suggest a possible role of immune mechanisms in the development of the disease, that may be associated in some cases with autoimmune disorders. To our knowledge 31 cases of association between thrombotic thrombocytopenic purpura and systemic lupus erythematosus are reported in the English literature from 1970 today; the link between the two diseases is unclear. The authors review these cases with particular care to the diagnosis, that may be very difficult, and the therapy.