1 Introduction Thrombotic thrombocytopenic purpura (TTP) is clinically characterized by the occurrence of thrombocytopenia and microangiopathic hemolytic anemia [ 1 , 2 , 3 ]. TTP was first described as a pathological entity in 1924 by Moschcowitz [ 1 ] and was clearly identified as an autoimmune disorder by Harrington et al. in 1951 [ 4 ]. Currently, after 70 years, we know that the majority of TTP patients suffer from acquired TTP caused by the presence of autoantibodies (AAbs) against ADAMTS13 [ 5 , 6 , 7 ], a protease that cleaves the von Willebrand
Hovinga JA, Lammle B. Role of ADAMTS13 in the pathogenesis, diagnosis, and treatment of thrombotic thrombocytopenic purpura. Hematology Am Soc Hematol Educ Program. 2012;2012(1):610-6. 6. Giblin JP, Hewlett LJ, Hannah MJ. Basal secretion of von Willebrand factor from human endothelial cells. Blood. 2008;112(4):957-964. 7. Springer TA. Biology and physics of von Willebrand factor concatamers. J Thromb Haemost. 2011;9(Suppl 1):130-143. 8. Zhou YF, Eng ET, Zhu J, et al. Sequence and structure relationships within von Willebrand factor. Blood. 2012;120(2):449-458. 9. Zhang
Med. 2002; 347: 589-600. 6. Lowe E, Werner E. Thrombotic thrombocitopenic purpura and hemolytic-uremic syndrome in children and adolescents. Sem Thromb Haem. 2005; 31: 717-29. 7. Nolasco L, Turner N, Bernardo A, et al. Hemolytic-uremic syndrome-associated Shiga toxins promote endothelial-cell secretion and impair ADAMTS13 cleavage of unusually large von Willebrand factor multimers. Blood. 2005; 106: 4199-209. 8. Mannucci PM, Canciani MT, Forza I, et al. Changes in health and disease of the metalloprotease that cleaves von Willebrand factor. Blood. 2001; 98: 2730
Acute kidney damage associated with pregnancy occurs in 1/20.000 pregnancies. In developing countries, the main cause of the development of acute kidney damage is septic abortion, and preeclampsia in the developed countries of the world. Preeclampsia is defined as newly developed hypertension, proteinuria and swelling in pregnant women after the 20th week of gestation. It occurs due to disorders in the development of placenta and systemic disorders of the function of the endothelium of the mother. It is treated with methyldopa, magnesium sulfate and timely delivery. Urgent delivery is indicated if the age of gestation is ≥ 34 weeks. HELLP syndrome is a difficult form of preeclampsia. Its main characteristics are decreased platelet count, microangiopathic hemolysis anemia, increased concentration of aminotransferase in the serum and acute kidney damage. Severe HELLP syndrome is treated with emergency delivery, antihypertensives, magnesium sulfate, and in some cases plasmapheresis and hemodialysis. Acute fatty liver in pregnancy occurs because of decreased activity of the LCHAD enzyme of the fetus. Due to the reduced beta oxidation of fatty acids in the hepatocytes of the fetus, long chain fatty acids that cause damage to the mother’s hepatocytes are released. Swansea criteria are used for diagnosis, and the difficult form of the disease is treated with plasmapheresis and extracorporeal liver support. Atypical HUS is due to a reduced protein activity that regulates the activity of the alternative pathway of the complement system. Its main features are thrombocytopenia, microangiopathic hemolytic anemia and acute kidney damage. It is treated with plasmapheresis, and in case of resistance with eculizumab. Thrombotic thrombocytopenic purpura is due to decreased activity of the ADAMTS13 enzyme. It is characterized by thrombocytopenia, microangiopathic hemolytic anemia, high temperature, nervous system disorders and acute kidney damage. It is treated with plasmapheresis, and severe form of disease with corticosteroids and azathioprine. Early detection and timely treatment of acute kidney damage provides a good outcome for the mother and fetus.
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