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. 2021 Jul 23;93(7):826-829.
doi: 10.26442/00403660.2021.07.200914.

[Treatment of thrombotic thrombocytopenic purpura]

[Article in Russian]
G M Galstyan  1 , A A Maschan  2 , E E Klebanova  1 , I I Kalinina  2
Affiliations

[Treatment of thrombotic thrombocytopenic purpura]

[Article in Russian]
G M Galstyan et al. Ter Arkh. .

Abstract
in English, Russian

The review discusses approaches to treatment of congenital thrombotic thrombocytopenic purpura (TTP) or Upshaw-Schulman syndrome. In congenital TTP, plasma transfusions are sufficient. Such treatment options as plasma exchange, administration of clotting factor VIII concentrate, recombinant ADAMTS13, are also used. Separately discussed issues of management of patients with TTP during pregnancy, and pediatric patients with TTP.

В обзоре обсуждаются подходы к лечению врожденной тромботической тромбоцитопенической пурпуры (ТТП), или синдрома АпшоуШульмана. При врожденной ТТП достаточно выполнение трансфузий плазмы, используются также такие методы лечения, как терапевтический плазмообмен, введение концентрата фактора свертывания VIII, рекомбинантного ADAMTS13. Отдельно обсуждаются вопросы ведения пациенток с ТТП во время беременности, особенности лечения ТТП у детей.

Keywords: ADAMTS13; Upshaw–Schulman syndrome; pregnancy; recombinant ADAMTS13; therapeutic plasma exchange; thrombotic thrombocytopenic purpura.

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