The acquired form of ADAMTS-13 deficiency as the cause of thrombotic microangiopathy in a pregnant woman with recurrent cerebral circulation disorders, venous thromboembolism, preeclampsia and fetal loss syndrome

Abstract

Thrombotic microangiopathy (TMA) is one of the most difficult thrombotic complications, characterized by damage of microvessels of various organs and accompanied by thrombocytopenia and hemolytic anemia. Today, TMA includes thrombotic thrombocytopenic purpura, hemolytic-uremic syndrome, heparin-induced thrombocytopenia and hemolysis, elevated liver enzymes and low platelet count (HELLP) syndrome. A key characteristic in the development of TMA is endothelial dysfunction. Recent discoveries suggest that this pathology is more widespread than it was previously. One of the most important triggers for TMA is pregnancy. The clinical observation of a patient with an inhibitory form of a disintegrin and metalloproteinase with a thrombospondrin type 1 motif, member 13 (ADAMTS-13) deficiency, recurrent thrombotic and "typical obstetric" severe complications is presented. We diagnosed a reduction in the activity of ADAMTS-13, an increase in the level of antibodies to ADAMTS-13 and disturbances in functioning of the protein C system along with symptoms of disorders of blood flow in the mother-placenta-fetus system as a poor prognosis in regard to both thrombotic and placenta-mediated combinations. An analysis of cases such as this patient immediately pushes the clinician to the idea of antiphospholipid syndrome (APS). It is questionable whether this patient could be regarded as an APS patient if her symptoms do not correspond with the classic diagnostic criteria for the syndrome. At the same time, the discovery of molecular mechanisms of TMA warrants a fresh look into the pathogenesis of thrombotic complications associated with pregnancy, as well as the pathogenesis of placental obstetric complications, including severe forms of preeclampsia, premature detachment of normally situated placenta and septic shock.