As recently written by Cruz and coll., antiphospholipid antibodies (aPL) are frequently
found in immune thrombocytopenia (ITP) and are not associated with specific clinical
feature [
[1]
]. Similarly, thrombocytopenia is frequently reported in APS patients and is one of
the most common non-criteria manifestations of the disease. Underlining pathological
mechanisms of thrombocytopenia in APS are various, and mostly not related to definite
ITP. In the life-threatening variant of APS, called the catastrophic APS (CAPS), thrombocytopenia
is due to a thrombotic microangiopathy characterized by the occurrence of thrombosis
in multiple organs over a short period of time [
[2]
]. Outside this variant, thrombocytopenia is often mild (between 100 and 150 × 109/L). The prevalence of thrombocytopenia in APS was estimated between 20 and53% [
- Rodríguez-Pintó I.
- Moitinho M.
- Santacreu I.
- Shoenfeld Y.
- Erkan D.
- Espinosa G.
- et al.
CAPS registry project group (European forum on antiphospholipid antibodies). catastrophic
antiphospholipid syndrome (CAPS): descriptive analysis of 500 patients from the international
caps registry.
Autoimmun Rev. 2016; 15: 1120-1124
[3]
]. However, previous studies were heterogeneous, included both primary and auto-immune-associated
APS, various definition of thrombocytopenia and/or patients with CAPS. The meaning
of low platelet count in primary APS remains unclear in patients without CAPS.To read this article in full you will need to make a payment
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References
- Clinical course of primary immune thrombocytopenia with positive antiphospholipid antibodies.Eur J Intern Med. 2019; 69: e6-e7
- CAPS registry project group (European forum on antiphospholipid antibodies). catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of 500 patients from the international caps registry.Autoimmun Rev. 2016; 15: 1120-1124
- The significance and management of thrombocytopenia in antiphospholipid syndrome.CurrRheumatol Rep. 2015; 17: 14
- International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS).J Thromb Haemost. 2006; 4: 295-306
- EULAR recommendations for the management of antiphospholipid syndrome in adults.Ann Rheum Dis. 2019; 78: 1296-1304
- Subcommittee on control of anticoagulation of the scientific and standardization committee of the international society on thrombosis and haemostasis. definition of major bleeding in clinical investigations of antihemostatic medicinal products in non-surgical patients.J Thromb Haemost. 2010; 8: 202-204
- GAPSS: the global anti-phospholipid syndrome score.Rheumatology (Oxford). 2013; 52: 1397-1403
Article info
Publication history
Published online: February 05, 2020
Accepted:
January 23,
2020
Received in revised form:
January 15,
2020
Received:
January 6,
2020
Identification
Copyright
© 2020 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.