Immune Thrombocytopenia in Antiphospholipid Syndrome: Is It Primary or Secondary?
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 th...
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doaj-b9dc35acc831466391fba45c1ce5c5bd2021-09-25T23:46:35ZengMDPI AGBiomedicines2227-90592021-09-0191170117010.3390/biomedicines9091170Immune Thrombocytopenia in Antiphospholipid Syndrome: Is It Primary or Secondary?Riccardo Tomasello0Giulio Giordano1Francesco Romano2Federica Vaccarino3Sergio Siragusa4Alessandro Lucchesi5Mariasanta Napolitano6Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties (ProMISE), University of Palermo, 90141 Palermo, ItalyDivision of Internal Medicine, Hematology Service, Regional Hospital “A. Cardarelli”, 86100 Campobasso, ItalyDepartment of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties (ProMISE), University of Palermo, 90141 Palermo, ItalyDepartment of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties (ProMISE), University of Palermo, 90141 Palermo, ItalyDepartment of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties (ProMISE), University of Palermo, 90141 Palermo, ItalyHaematology Unit, IRCCS Istituto Romagnolo per lo Studio dei Tumori “Dino Amadori” (IRST), 47104 Meldola, ItalyDepartment of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties (ProMISE), University of Palermo, 90141 Palermo, ItalyAntiphospholipid 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.https://www.mdpi.com/2227-9059/9/9/1170antiphospholipid antibodiesantiphospholipid syndromethrombocytopenialupus anticoagulantimmune thrombocytopenia |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Riccardo Tomasello Giulio Giordano Francesco Romano Federica Vaccarino Sergio Siragusa Alessandro Lucchesi Mariasanta Napolitano |
spellingShingle |
Riccardo Tomasello Giulio Giordano Francesco Romano Federica Vaccarino Sergio Siragusa Alessandro Lucchesi Mariasanta Napolitano Immune Thrombocytopenia in Antiphospholipid Syndrome: Is It Primary or Secondary? Biomedicines antiphospholipid antibodies antiphospholipid syndrome thrombocytopenia lupus anticoagulant immune thrombocytopenia |
author_facet |
Riccardo Tomasello Giulio Giordano Francesco Romano Federica Vaccarino Sergio Siragusa Alessandro Lucchesi Mariasanta Napolitano |
author_sort |
Riccardo Tomasello |
title |
Immune Thrombocytopenia in Antiphospholipid Syndrome: Is It Primary or Secondary? |
title_short |
Immune Thrombocytopenia in Antiphospholipid Syndrome: Is It Primary or Secondary? |
title_full |
Immune Thrombocytopenia in Antiphospholipid Syndrome: Is It Primary or Secondary? |
title_fullStr |
Immune Thrombocytopenia in Antiphospholipid Syndrome: Is It Primary or Secondary? |
title_full_unstemmed |
Immune Thrombocytopenia in Antiphospholipid Syndrome: Is It Primary or Secondary? |
title_sort |
immune thrombocytopenia in antiphospholipid syndrome: is it primary or secondary? |
publisher |
MDPI AG |
series |
Biomedicines |
issn |
2227-9059 |
publishDate |
2021-09-01 |
description |
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. |
topic |
antiphospholipid antibodies antiphospholipid syndrome thrombocytopenia lupus anticoagulant immune thrombocytopenia |
url |
https://www.mdpi.com/2227-9059/9/9/1170 |
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