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

Catastrophic antiphospholipid syndrome (Ronald Asherson syndrome) and obstetric pathology

Alexander D Makatsariya  1 , Jamilya Khizroeva  2 , Viktoriya O Bitsadze  2
Affiliations

Catastrophic antiphospholipid syndrome (Ronald Asherson syndrome) and obstetric pathology

Alexander D Makatsariya et al. J Perinat Med. .

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%.

Keywords: Antiphospholipid syndrome; catastrophic APS; rare disease; thrombotic storm.

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References

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