Efficacy of plasma exchange in the therapy of recurrent pregnancy-associated atypical hemolytic-uremic syndrome
Background. Pregnancy-associated atypical hemolytic-uremic syndrome (aHUS) is characterized by an aggressive avalanche-like course and an extremely poor prognosis with fulminant thrombotic microangiopathy (TMA), whereas targeted therapy with a complement-blocking drug is always impossible in the first 24 hours. There are very few cases of recurrent pregnancy-associated aHUS in the modern literature; in this connection it is important to display the management of patients with the second disease episode that has developed during pregnancy. In the era of eculizumab, the role of plasma exchange in verified aHUS is becoming increasingly limited. Just the same, when eculizumab is not available, plasma exchange remains the only treatment option.Kirsanova T.V., Fedorova T.A., Gurbanova S.R., Pyregov A.V., Vinogradova M.A.
Case report. The paper presents the experience in successfully treating two consecutive pregnancy-associated aHUS episodes manifested after delivery in one patient, by using only plasma exchange, which showed a favorable outcome, including complete recovery of renal function.
Conclusion. Different types of TMA in pregnancy are fulminant and represent a life-threatening condition for the mother. In this condition, the most urgent issue is the timely initiation of appropriate therapy. The presented observation of two aHUS episodes demonstrates difficulties in choosing a treatment policy and in making a rapid differential diagnosis, even when there is only one episode of TMA in the history. The discussion lists the recommended treatment regimens, as well as the possibility of a favorable outcome with timely initiation of plasma therapy
Keywords
plasma exchange
thrombotic microangiopathy
atypical hemolytic-uremic syndrome (a-HUS)
pregnancy
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Received 02.12.2020
Accepted 29.04.2021
About the Authors
Tatyana V. Kirsanova, PhD, Senior Researcher of the Department of Transfusiology and Extracorporeal Hemocorrection, Academician V.I. Kulakov National Medical Research Center for Obstetrics, Gynecology and Perinatology, Ministry of Health of Russia. Tel.: +7(926)248-45-60. E-mail: a_tatya@mail.ru. ORCID: 0000-0002-6125-590X.117997, Russia, Moscow, Academician Oparin str., 4.
Tatiana A. Fedorova, MD, professor, Head of the Department of Transfusiology and Extracorporeal Hemocorrection, Academician V.I. Kulakov National Medical Research Center for Obstetrics, Gynecology and Perinatology, Ministry of Health of Russia. E-mail: tfedorova1@mail.ru. ORCID: 0000-0001-6883-4456.
117997, Russia, Moscow, Academician Oparin str., 4.
Samira R. Gurbanova, PhD, researcher of the Department of Extracorporal Methods of Treatment and Detoxification, Academician V.I. Kulakov National Medical Research Center for Obstetrics, Gynecology and Perinatology, Ministry of Health of Russia. E-mail: gursam81@mail.ru. ORCID: 0000-0003-3491-1868.
117997, Russia, Moscow, Academician Oparin str., 4.
Maria A. Vinogradova, PhD, Head of Department of Reproductive Hematology and Clinical Hemostasiology, Academician V.I. Kulakov National Medical Research
Center for Obstetrics, Gynecology and Perinatology, Ministry of Health of Russia. E-mail: mary-grape@yandex.ru. ORCID: 0000-0001-9827-1922.
117997, Russia, Moscow, Academician Oparin str., 4.
Aleksey V. Pyregov, MD, Head of the Department of Anesthesiology-Resuscitation, Academician V.I. Kulakov National Medical Research Center for Obstetrics, Gynecology and Perinatology, Ministry of Health of Russia. E-mail: a_pyregov@oparina4.ru. ORCID: 0000-0001-8382-9671. 117997, Russia, Moscow, Academician Oparin str., 4.
For citation: Kirsanova T.V., Fedorova T.A., Gurbanova S.R., Pyregov A.V., Vinogradova M.A. Efficacy of plasma exchange in the therapy of recurrent
pregnancy-associated atypical hemolytic-uremic syndrome.
Akusherstvo i Ginekologiya/Obstetrics and Gynecology. 2021; 6: 186-191 (in Russian)
https://dx.doi.org/10.18565/aig.2021.6.186-191
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