灾难性抗磷脂综合征（罗纳德·阿舍尔森综合征）与产科病理学

Catastrophic antiphospholipid syndrome (Ronald Asherson syndrome) and obstetric pathology.

作者信息

Makatsariya Alexander D, Khizroeva Jamilya, Bitsadze Viktoriya O

机构信息

Correspondent Member of the Russian Academy of Sciences, Head of the Chair of Obstetrics and Gynecology of Medical Prophylaxis Faculty of I.M. Sechenov First Moscow State Medical University, Vice-President of Russian Association of Obstetricians and Gynaecologists, Dmitrii Ulyanov Street 4-2-432, 119333 Moscow, Russia, Tel.: +7(903)728-0897.

Professors of the Chair of Obstetrics and Gynecology of Medical Prophylaxis Faculty of I.M. Sechenov First Moscow State Medical University, Zemlyanoi Val, 62, 109004 Moscow, Russia.

出版信息

J Perinat Med. 2018 May 24;46(4):387-400. doi: 10.1515/jpm-2017-0092.

DOI:10.1515/jpm-2017-0092

PMID:28829758

Abstract

BACKGROUND

Catastrophic antiphospholipid syndrome (CAPS) is an uncommon, often fatal, variant of the antiphospholipid syndrome (APS) that results in a widespread coagulopathy and high titres of antiphospholipid antibodies (aPL) and affects predominantly small vessels supplying organs with the development of multiorgan failure. It remains unclear why some patients develop the typical clinical picture of APS (thrombosis of large vessels), whereas others show the development of progressive microthrombosis, which the authors called "thrombotic storm" and multiple organ failure, that is, CAPS.

MATERIALS AND METHODS

Since 2001-2016, we discovered 17 patients with CAPS development.

CONCLUSION

CAPS is life-threatening condition, but optimal treatment for CAPS is not developed yet and the mortality rate is as high as 30%-40%.

摘要

背景

灾难性抗磷脂综合征（CAPS）是抗磷脂综合征（APS）的一种罕见且通常致命的变体，它会导致广泛的凝血病和高滴度的抗磷脂抗体（aPL），主要影响为器官供血的小血管，进而发展为多器官功能衰竭。目前尚不清楚为何有些患者会出现典型的APS临床表现（大血管血栓形成），而另一些患者则表现为进行性微血栓形成，即作者所称的“血栓风暴”以及多器官功能衰竭，也就是CAPS。