Diagnosis and management of catastrophic antiphospholipid syndrome. Issue 4 (3rd April 2017)

• Record Type: Journal Article
• Authors: Carmi, Or; Berla, Maya; Shoenfeld, Yehuda; Levy, Yair
• Is Part Of: Expert review of hematology. Volume 10:Issue 4(2017)
• Page Start: 365
• Page End: 374
• Publication Date: 2017-04-03
• Subjects: Antiphospholipid antibodies -- catastrophic antiphospholipid syndrome -- hemolytic anemia -- lupus anticoagulant -- thrombotic microangiopathic hemolytic anemia; Hematology -- Periodicals; 616.15005
• DOI: 10.1080/17474086.2017.1300522
• Languages: English
• ISSNs: 1747-4086

Abstract

ABSTRACT: Introduction : Catastrophic antiphospholipid syndrome (CAPS) is a rare, life-threatening disease. In 1992, Asherson defined it as a widespread coagulopathy related to the antiphospholipid antibodies (aPL). CAPS requires rapid diagnosis and prompt initiation of treatment. Areas covered : This paper discusses all aspects of CAPS, including its pathophysiology, clinical manifestations, diagnostic approaches, differential diagnoses, management and treatment of relapsing CAPS, and its prognosis. To obtain the information used in this review, scientific databases were searched using the key words antiphospholipid antibodies, catastrophic antiphospholipid syndrome, hemolytic anemia, lupus anticoagulant, and thrombotic microangiopathic hemolytic anemia. Expert commentary : CAPS is a rare variant of the antiphospholipid syndrome (APS). It is characterized by thrombosis in multiple organs and a cytokine storm developing over a short period, with histopathologic evidence of multiple microthromboses, and laboratory confirmation of high aPL titers. This review discusses the diagnostic challenges and current approaches to the treatment of CAPS.