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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

2010; 116 : 4060-4069. 5. VESELY SK, GEORGE JN, LAMMLE B, STUDT JD, ALBERIO L, EL- HARAKE MA, et al. ADAMTS13 activity in thrombotic thrombocytopenic purpura- hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients. Blood 2003; 102 : 60-68. 6. GADDAM S, PABLANI L, CHAINANI V, KAVUDA RR, NAGRANI T, ABOU RJAILI G, et al. Complete recovery of ischemic cardiomyopathy from thrombotic thrombocytopenic purpura . Clinical Medicine Insights Cardiology 2011; 5 : 29-33. 7. HAWKINS BM, ABU-FADEL M, VESELY SK

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.

References 1. Bryckaert M, Rosa JP, Denis CV, Lenting PJ- Of von Willebrand factor and platelets. Cell Mol Life Sci. 2015;72:307-326. 2. McKinnon TA, Chion AC, Millington AJ, Lane DA, Laffan MA- N-linked glycosylation of VWF modulates its interaction with ADAMTS13. Blood. 2008;111:3042-3049. 3. Reid P, Holen I – Pathophysiological role of osteoprotegerin (OPG). European Journal of Cell Biology. 2009;88:1-17. 4. Shahbazi S, Lenting PJ, Fribourg C, Terraube V, Denis CV, Christophe OD - Characterization of the interaction between von Willebrand factor and

and proteolysis. 1999; 13(6):239-244 19. Andersson HM, Siegerink B, Luken BM, Crawley JT, Algra A, Lane DA, Rosendaal FR. High VWF, low ADAMTS13, and oral contraceptives increase the risk of ischemic stroke and myocardial infarction in young women. Blood. 2012 Feb 9;119(6):1555-60.

alleles are unlikely to contribute as strongly as the FV and ABO loci to VTE risk: results from a GWAS approach. Blood 2009; 113: 5298-303. 40. McGrath RT, McKinnon TA, Byrne B, O'Kennedy R, Terraube V, McRae E, et al. Expression of terminal alpha2- 6-linked sialic acid on von Willebrand factor specifically enhances proteolysis by ADAMTS13. Blood 2010; 115: 2666-73. 41. Ohira T, Cushman M, Tsai MY, Zhang Y, Heckbert SR, Zakai NA, et al. ABO blood group, other risk factors and incidence of venous thromboembolism: the Longitudinal Investigation of Thromboembolism Etiology

. 2014 93 2 103 111 19 Bowen DJ, Collins PW. An amino acid polymorphism in von willebrand factor correlates with increased susceptibility to proteolysis by ADAMTS13. Blood. 2004; 103(3): 941-947. 10.1182/blood-2003-05-1505 Bowen DJ Collins PW. An amino acid polymorphism in von willebrand factor correlates with increased susceptibility to proteolysis by ADAMTS13 Blood. 2004 103 3 941 947 20 van ’t Hooft FM, Silveira A, Tornvall P, Iliadou A, Ehrenborg E, Eriksson P, et al . Two common functional polymorphisms in the promoter region of the coagulation factor VII gene

Biol 2007; 27: 955-62. Leebeek FW, Goor MP, Guimaraes AH, Brouwers GJ, Maat MP, Dippel DW, Rijken DC. High functional levels of thrombin-activatable fibrinolysis inhibitor are associated with an increased risk of first ischemic stroke. J Thromb Haemost 2005; 3: 2211-8. Bongers TN, de Maat MP, van Goor ML, Bhagwanbali V, van Vliet HH, Gomez Garcia EB, Dippel DW, Leebeek FW. High von Willebrand factor levels increase the risk of first ischemic stroke: influence of ADAMTS13, inflammation, and genetic variability. Stroke 2006; 37: 2672-7. Dai K, Gao W, Ruan C. The