Immune Thrombocytopenia in Antiphospholipid Syndrome: Is It Primary or Secondary?

Biomedicines

Riccardo Tomasello¹  Federica Vaccarino¹  Mariasanta Napolitano¹  Sergio Siragusa¹  Francesco Romano¹  Giulio Giordano²  Alessandro Lucchesi³ 

[1] Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties (ProMISE), University of Palermo, 90141 Palermo, Italy;Division of Internal Medicine, Hematology Service, Regional Hospital “A. Cardarelli”, 86100 Campobasso, Italy;Haematology Unit, IRCCS Istituto Romagnolo per lo Studio dei Tumori “Dino Amadori” (IRST), 47104 Meldola, Italy;

关键词: antiphospholipid antibodies;    antiphospholipid syndrome;    thrombocytopenia;    lupus anticoagulant;    immune thrombocytopenia;   

DOI  :  10.3390/biomedicines9091170

来源: DOAJ

【 摘 要 】

Antiphospholipid syndrome (APS) is frequently associated with thrombocytopenia, in most cases mild and in the absence of major bleedings. In some patients with a confirmed APS diagnosis, secondary immune thrombocytopenia (ITP) may lead to severe thrombocytopenia with consequent major bleeding. At the same time, the presence of antiphospholipid antibodies (aPL) in patients with a diagnosis of primary ITP has been reported in several studies, although with some specific characteristics especially related to the variety of antigenic targets. Even though it does not enter the APS defining criteria, thrombocytopenia should be regarded as a warning sign of a “high risk” APS and thus thoroughly evaluated. The presence of aPL in patients with ITP should be assessed as well to stratify the risk of paradoxical thrombosis. In detail, besides the high hemorrhagic risk in secondary thrombocytopenia, patients with a co-diagnosis of APS or only antibodies are also at risk of arterial and venous thrombosis. In this narrative review, we discuss the correlation between APS and ITP, the mechanisms behind the above-reported entities, in order to support clinicians to define the most appropriate treatment strategy in these patients, especially when anticoagulant or antiplatelet agents may be needed.

【 授权许可】

Unknown