THE IMITATORS OF SEVERE PREECLAMPSIA AND HELLP SYNDROME: DIFFERENT TYPES OF PREGNANCY-ASSOCIATED THROMBOTIC MICROANGIOPATHY


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Abstract

Objective. To carry out a systematic analysis of the data available in the current literature on states that can imitate preeclampsia, in the genesis of which pregnancy will play not a major role, but a trigger one. Material and methods. The review included the data of foreign and Russian papers published in the past 5 years and found in Pubmed on this topic. Results. The paper describes a differential diagnostic search between the different types of thrombotic microangiopathy (TMA), which can be induced by pregnancy since a correct diagnosis frequently affects life expectancy primarily in a mother and her child. Thrombotic thrombocytopenic purpura and atypical hemolytic-uremic syndrome are life-threatening conditions during pregnancy and require timely adequate treatment and further follow-up. HELLP-syndrome and preeclampsia have similar clinical characteristics. Conclusion. The main differences between preeclampsia and HELLP syndrome, on the one hand, and life-threatening conditions, such as atypical hemolytic-uremic syndrome, thrombotic thrombocytopenic purpura, and catastrophic antiphospholipid syndrome, on the other hand, are considered. An algorithm of diagnostic search for pregnancy-associated TMA is proposed, key differences determined, and therapeutic approaches approached.

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About the authors

Tatyana Valeryevna Kirsanova

Email: A_Tatya@mail.ru
PhD, senior researcher of the Department of Reproductive Hematology and Clinical Hemostasis, Research Center of Obstetrics, Gynecology and Perinatology, Ministry of Health of Russia 117997, Russia, Moscow, Ac. Oparina str. 4

Maria Alekseevna Vinogradova

Email: mary-grape@ya.ru
PhD, head of Department of Reproductive Hematology and Clinical Hemostasis, Research Center of Obstetrics, Gynecology and Perinatology, Ministry of Health of Russia. 117997, Russia, Moscow, Ac. Oparina str. 4

Tatyana Anatolyevna Fedorova

Email: tfedorova1@mail.ru
Professor, MD, the Chief of Department of Transfusiology and Extracorporeal Haemocorrection, Research Center of Obstetrics, Gynecology and Perinatology, Ministry of Health of Russia. 117997, Russia, Moscow, Ac. Oparina str. 4

References

  1. Weinstein L. Syndrome of hemolysis, elevated liver enzymes, and low platelet count: a severe consequence of hypertension in pregnancy. Am. J. Obstet. Gynecol. 1982; 142(2): 159-67.
  2. Sibai B.M., Ramadan M.K., Usta I., Salama M., Mercer B.M., Friedman S.A. Maternal morbidity and mortality in 442 pregnancies with hemolysis, elevated liver enzymes, and low platelets (HELLP syndrome). Am. J. Obstet. Gynecol. 1993: 169(4): 1000-6.
  3. Sibai B.M., Ramadan M.K., Chari R.S., Friedman S.A. Pregnancies complicated by HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets): subsequent pregnancy outcome and long-term prognosis. Am. J. Obstet. Gynecol. 1995; 172(1, Pt 1): 125-9.
  4. Sibai B.M., Ramadan M.K. Acute renal failure in pregnancies complicated by hemolysis, elevated liver enzymes, and low platelets. Am. J. Obstet. Gynecol. 1993; 168(6, Pt 1): 1682-7.
  5. Lesesve J.-F., Fenneteau O., Cynober T., Lecompte T., Grange M.-J., Flandrin G., Troussard X. GFHC Rôle du biologiste confronté à la recherche de schizocytes Texte de synthèse et de recommandations du Groupe français d’hématologie cellulaire (GFHC). Ann. Biol. Clin. 2003; 61(5): 505-12.
  6. Burns E.R., Lou Y., Pathak A. Morphologic diagnosis of thrombotic thrombocytopenic purpura. Am. J. Hematol. 2004; 75(1): 18-21.
  7. Dusse L.M., Alpoim P.N., Silva J.T., Rios D.R., Brandäo A.H., Cabral A.C. Revisiting HELLP syndrome. Clin. Chim. Acta. 2015; 451(Pt B): 117-20.
  8. George J.N. How I treat patients with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Blood. 2000; 96(4): 1223-9.
  9. Zini G., d’Onofrio G., Briggs C., Erber W., Jou J.M., Lee S.H. et al. ICSH recommendations for identification, diagnostic value, and quantitation of schistocytes. Int. J. Lab. Hematol. 2012; 34(2): 107-16.
  10. Fakhouri F., Roumenina L., Provot F., Sallée M., Caillard S., Couzi L. et al. Pregnancy-associated hemolytic uremic syndrome revisited in the era of complement gene mutations. J. Am. Soc. Nephrol. 2010; 21(5): 859-67.
  11. Noris M., Perico N., Remuzzi G. Mechanisms of disease: pre-eclampsia. Nat. Clin. Pract. Nephrol. 2005; 1(2): 94, 98-114.
  12. Fakhouri F., Vercel C., Frémeaux-Bacchi V. Obstetric nephrology: AKI and thrombotic microangiopathies in pregnancy. Clin. J. Am. Soc. Nephrol. 2012; 7(12): 2100-6.
  13. George J.N. Evaluation of patients with microangiopathic hemolytic anemia and thrombocytopenia. Semin. Thromb. Hemost. 2013; 39(2): 153-60.
  14. Hassan S., Westwood J.P., Ellis D., Laing C., Mc Guckin S., Benjamin S., Scully M. The utility of ADAMTS13 in differentiating TTP from other acute thrombotic microangiopathies: results from the UK TTP Registry. Br. J. Haematol. 2015; 171(5): 830-5.
  15. Bendapudi P.K., Li A., Hamdan A., Uhl L., Kaujman R., Stowell C. et al. Impact of severe ADAMTS13 deficiency on clinical presentation and outcomes in patients with thrombotic microangiopathies: the experience of the Harvard TMA Research Collaborative. Br. J. Haematol. 2015; 171(5): 836-44.
  16. Martin J.N. Jr., Bailey A.P., Rehberg J.F., Owens M.T., Keiser S.D., May W.L. Thrombotic thrombocytopenic purpura in 166 pregnancies: 1955-2006. Am. J. Obstet. Gynecol. 2008; 199(2): 98-104.
  17. Keiser S.D., Boyd K.W., Rehberg J.F., Elkins S., Owens M.Y., Sunesara I., Martin J.N. Jr. A high LDH to AST ratio helps to differentiate pregnancy-associated thrombotic thrombocytopenic purpura (TTP) from HELLP syndrome. J. Matern. Fetal Neonatal Med. 2012; 25(7): 1059-63.
  18. Fujimura Y., Matsumoto M., Kokame K., Isonishi A., Soejima K., Akiyama N. et al. Pregnancy-induced throm-bocytopenia and TTP, and the risk of fetal death, in Upshaw-Schulman syndrome: A series of 15 pregnancies in 9 genotyped patients. Br. J. Haematol. 2009; 144(5): 742-54.
  19. Fakhouri F., Fremeaux-Bacchi V. Does hemolytic uremic syndrome differ from thrombotic thrombocytopenic purpura. Nat. Clin. Pract. Nephrol. 2007; 3(12): 679-87.
  20. Noris M., Caprioli J., Bresin E., Mossali C., Pianetti G., Gamba S. et al. Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype. Clin. J. Am. Soc. Nephrol. 2010; 5(10): 1844-59.
  21. Noris M., Remuzzi G. Atypical hemolytic-uremic syndrome. N. Engl. J. Med. 2009; 361(17): 1676-87.
  22. Козловская Н.Л., Меркушева Л.И., Кирсанова Т.В., Рунихина Н.К. Особенности течения и исхода атипичного гемолитико-уремического синдрома при беременности. Клиническая нефрология. 2012; 3: 44-9. [Kozlovskaya N.L., Merkusheva L.I., Kirsanova T.V., Runihina N.K. Features of the course and outcome of atypical hemolytic uremic syndrome in pregnancy. Klinicheskaya nefrologiya. 2012; 3: 44-9.
  23. Riedl M., Fakhouri F., Le Quintrec M., Noone D.G., Jungraithmayr T.C., Fremeaux-Bacchi V., Licht C. Spectrum of complement-mediated thrombotic microangiopathies: pathogenetic insights identify ing novel treatment approaches. Semin. Thromb. Hemost. 2014; 40(4): 444-64.
  24. Alberti M., Valoti E., Piras R. Two patients with history of STEC-HUS, posttransplant recurrence and complement gene mutations. Am. J Transplant. 2013; 13(8): 2201-6.
  25. McMinn J.R., George J.N. Evaluation of women with clinically suspected thrombotic thrombocytopenic purpura-hemolytic uremic syndrome during pregnancy. J. Clin. Apher. 2001; 16(4): 202-9.
  26. Кирсанова Т.В., Виноградова М.А., Федорова Т.А. Атипичный гемолитико-уремический синдром, ассоциированный с беременностью. Гематология и трансфузиология. 2016; Приложение №1(1) (Материалы III конгресса гематологов России 14-16 апреля 2016 года): 49. [Kirsanova T.V., Vinogradova M.A., Fedorova T.A. Atypical hemolytic uremic syndrome associated with pregnancy. Hematology and Blood Transfusion. 2016; Appendix №1 (1) (Proceedings of the III Congress of Hematology Russia on April 14-16, 2016): 49. (in Russian)]
  27. Ganesan C., Maynard S.E. Acute kidney injury in pregnancy: the thrombotic microangiopathies. J. Nephrol. 2011; 24(5): 554-63.
  28. Yelnik C., Laskin C., Porter T.F., Branch D.W., Buyon J.P., Guerra M.M. et al. Lupus anticoagulant is the main predictor of adverse pregnancy outcomes in aPL-positive patients: validation of PROMISSE study results. Lupus Sci. Med. 2016; 3(1): e000131.
  29. Asherson R.A., Cervera R. Catastrophic antiphospholipid syndrome. Curr. Opin. Hematol. 2000; 7(5): 325-9.
  30. Cervera R., Bucciarelli S., Plasin M.A., Gomez-Puerta J.A., Plaza J., Pons-Estel G. et al. Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of a series of 280 patients from the «CAPS Registry». J. Autoimmun. 2009; 32(3-4): 240-5.
  31. Asherson R.A. Multiorgan failure and antiphospholipid antibodies: the catastrophic antiphospholipid (Ashersons) syndrome. Immunobiology. 2005; 210(10): 727-33.
  32. Kitchens C.S. Thrombotic storm: when thrombosis begets thrombosis. Am. J. Med. 1998; 104(4): 381-5.
  33. Levine R.J., Maynard S.E., Qian C., Lim K.H., England L.J., Yu K.F. et al. Circulating angiogenic factors and the risk of preeclampsia. N. Engl. J. Med. 2004; 350(7): 672-83.
  34. Козловская Н.Л., Меркушева Л.И., Кирсанова Т.В., Бобкова И.Н., Боброва Л.А., Рунихина Н.К. Влияние дисбаланса плацентарных факторов ангиогенеза на клинические проявления «ранней» и «своевременной» преэклампсии. Нефрология и диализ. 2013; 15(3): 206-15. [Kozlovskaya N.L., Merkusheva L.I., Kirsanova T.V., Bobkova I.N., Bobrova L.A., Runihina N.K. Influence of imbalance placental angiogenesis factors on the clinical manifestations of „early” and „timely” preeclampsia. Nefrologiya i dializ. 2013; 15(3): 206-15. (in Russian)]
  35. Stillman I.E., Karumanchi S.A. The glomerular injury of preeclampsia. J. Am. Soc. Nephrol. 2007; 18(8): 2281-4.
  36. Karumanchi S.A., Maynard S.E., Stillman I.E., Epstein F.H., Sukhatme V.P. Preeclampsia: a renal perspective. Kidney Int. 2005; 67(6): 2101-13.
  37. Haram K., Svendsen E., Abildgaard U. The HELLP syndrome: clinical issues and management. A review. BMC Pregnancy Childbirth. 2009; 9: 8.
  38. Barton J.R., Riely C.A., Adamec T.A., Shanklin D.R. et al. Hepatic histopathologic condition does not correlate with laboratory abnormalities in HELLP syndrome (hemolisis, elevated liver enzymes, and low platelet count). Am. J. Obstet. Gynecol. 1992; 167(6): 1538-43.
  39. Chawla S., Marwaha A., Agarwal R. HELLP or help: a real challenge. J. Obstet. Gynaecol. India. 2015; 65(3): 172-5.
  40. Van Dam P.A., Renier M., Baekelandt M., Buytaert P., Uyttenbroeck F. Disseminated intravascular coagulation and the syndrome of hemolysis, elevated liver enzymes, and low platelets in severe preeclampsia. Obstet. Gynecol. 1989; 73(1): 97-102.
  41. Sagawa N., Kariya M., Kanzaki H., Fujii S., Matsuura S., Mori T. A case of postpartum hemolytic uremic syndrome with severe elevations of liver enzymes. Obstet. Gynecol. 1985; 65(5): 761-4.
  42. Payne B.A., Hutcheon J.A., Ansermino J.M., Hall D.R., Bhutta Z.A., Bhutta S.Z. et al. A risk prediction model for the assessment and triage of women with hypertensive disorders of pregnancy in low-resourced settings: the miniPIERS (Pre-eclampsia Integrated Estimate of RiSk):multi-country prospective cohort study. PLoS Med. 2014; 11(1): e1001589.
  43. Fakhouri F., Jablonski M., Lepercq J., Blouin J., Benachi A., Hourmant M. et al. Factor H, membrane cofactor protein, and factor I mutations in patients with hemolysis, elevated liver enzymes, and low platelet count syndrome. Blood. 2008; 112(12): 4542-5.
  44. Crovetto F., Borsa N., Acaia B., Nishimura C., Frees K., Smith R.J. et al. The genetics of the alternative pathway of complement in the pathogenesis of HELLP syndrome. J. Matern. Fetal Neonatal Med. 2012; 25(11): 2322-5.
  45. Wu V.C., Lin S.L., Tsai C.C., Tien H.F. Postpartum hemolytic uremic syndrome following abruptio placenta: report of a case. J. Formos. Med. Assoc. 2002; 101(12): 868-70.
  46. Gallwas J., Ackermann H., Friedmann W. Thrombotic thrombocytopenic pur-pura-a rare and difficult differential diagnosis to HELLP syndrome in late pregnancy. Z. Geburtshilfe Neonatol. 2008; 212(2): 64-6.
  47. Zhao C., Qu Y., Sui R., Feng J., Gao J., Ma J., Jiang R., Li H. Delayed visual recovery in pregnancy-associated thrombotic thrombocytopenic purpura with bilateral serous retinal detachment. Doc. Ophthalmol. 2013; 126(2): 163-9.
  48. Coppo P., Schwarzinger M., Buffet M., Wynckel A., Clabault K., Presne C. et al. Predictive features of severe acquired ADAMTS13 deficiency in idiopathic thrombotic microangiopathies: the French TMA reference center experience. PLoS One. 2010; 5(4): e10208.
  49. Ferrari S., Scheiflinger F., Rieger M., Mudde G., Wolf M., Coppo P. et al. Prognostic value of anti-ADAMTS 13 antibody features (Ig isotype, titer, and inhibitory effect) in a cohort of 35 adult French patients undergoing a first episode of thrombotic microangiopathy with undetectable ADAMTS 13 activity. Blood. 2007; 109(7): 2815-22.
  50. Scully M., McDonald V., Cavenagh J., Hunt B.J., Longair I., Cohen H., Machin S.J. A phase 2 study of the safety and efficacy of rituximab with plasma exchange in acute acquired thrombotic thrombocytopenic purpura. Blood. 2011; 118(7): 1746-53.
  51. George J.N., Nester C.M., McIntosh J.J. Syndromes of thrombotic microangiopathy associated with pregnancy. Hematology Am. Soc. Hematol. Educ. Program. 2015; 2015: 644-8.
  52. Beucher G., Simonet T., Dreyfus M. Management of the HELLP syndrome. Gynecol. Obstet. Fertil. 2008; 36(12): 1175-90.
  53. Eser B., Guven M., Unal A., Coskun R., Altuntas F., Sungur M. et al. The role of plasma exchange in HELLP syndrome. Clin. Appl. Thromb. Hemost. 2005; 11(2): 211-7.
  54. Martin J.N. Jr. Milestones in the quest for best management of patients with HELLP syndrome (microangiopathic hemolytic anemia, hepatic dysfunction, thrombocytopenia). Int. J. Gynaecol. Obstet. 2013; 121(3): 202-7.
  55. Simetka O., Klat J., Gumulec J., Dolezalkova E., Salounova D. Early identification of women with HELLP syndrome who need plasma exchange after delivery. Transfusion and Apheresis Scienc 52 (2015): 54-59
  56. Kazzaz N.M., McCune W.J., Knight J.S. Treatment of catastrophic antiphospholipid syndrome. Curr Opin Rheumatol. 2016 May ;28(3):218

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