Effective treatment due to misconception in the diagnosis

The development of thrombotic microangiopathy (TMA) during pregnancy and in postpartum period requires differential diagnosis between classical obstetric TMA (preeclampsia and HELLP-syndrome) and TMA, for which pregnancy can become a trigger of atypical hemolytic-uremic syndrome (аHUS), thrombotic thrombocytopenic purpura (TTP), obstetric sepsis with DIC-syndrome and catastrophic antiphospholipid syndrome (CAPS) in order to determine treatment strategy. Complement blocking treatment with eculizumab requires aHUS diagnosis, in the pathogenesis of which the main role is played by the uncontrolled activation of the alternative complement pathway. Complement dysregulation occurs in TMAs different from primary aHUS. Short treatment with eculizumab can result in a rapid improvement of patients with secondary aHUS in whom TMA has persisted and renal function worsened despite treatment of the TMA-inducing condition. This observation demonstrates the first experience of successful postpartum catastrophic antiphospholipid syndrome (CAPS) treatment with complement-blocking drug ekulizumab in Russia. Underestimation of the importance of detected laboratory changes and information about a burdened obstetric anamnesis led to the mistaken diagnosis of atypical HUS, which served as the basis for the ekulizumab prescription, without a doubt, saved the patient's life. The role of the complement activation in the pathogenesis of CAPS is discussed. A conclusion is made that the use of complement-blocking therapy in combination with anticoagulants primarily is advised in the obstetric CAPS patients.

тромботическая микроангиопатия, катастрофический антифосфолипидный синдром, беременность, экулизумаб, thrombotic microangiopathy catastrophic antiphospholipid syndrome, pregnancy, eculizumab

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