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Thrombocytopenia in primary antiphospholipid syndrome, a marker of high-risk patients?

  • Cécile M. Yelnik
    Correspondence
    Corresponding author: Department of Internal Medicine, Hôpital Claude Huriez, rue Polonovski, 59037 Lille Cedex, France.
    Affiliations
    Univ. Lille, INSERM U995, CHU Lille, Département de Médecine Interne et d'Immunologie clinique, Centre National de Référence Maladies Systémiques et Auto-Immunes Rares, European Reference Network on Rare Connective Tissue and MusculoskeletalDiseases (ReCONNECT), F-59000 Lille, France
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  • Yann Nguyen
    Affiliations
    Université Paris Descartes-Sorbonne Paris Cité, INSERM U1153, Service de Médecine Interne, Centre de Référence des Maladies Systémiques et Auto-Immunes Rares, Hôpital Cochin, F-75000 Paris, France
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  • Véronique Le Guern
    Affiliations
    Université Paris Descartes-Sorbonne Paris Cité, INSERM U1153, Service de Médecine Interne, Centre de Référence des Maladies Systémiques et Auto-Immunes Rares, Hôpital Cochin, F-75000 Paris, France
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  • Eric Hachulla
    Affiliations
    Univ. Lille, INSERM U995, CHU Lille, Département de Médecine Interne et d'Immunologie clinique, Centre National de Référence Maladies Systémiques et Auto-Immunes Rares, European Reference Network on Rare Connective Tissue and MusculoskeletalDiseases (ReCONNECT), F-59000 Lille, France
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  • Marc Lambert
    Affiliations
    Univ. Lille, INSERM U995, CHU Lille, Département de Médecine Interne et d'Immunologie clinique, Centre National de Référence Maladies Systémiques et Auto-Immunes Rares, European Reference Network on Rare Connective Tissue and MusculoskeletalDiseases (ReCONNECT), F-59000 Lille, France
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Published:February 05, 2020DOI:https://doi.org/10.1016/j.ejim.2020.01.017
      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 [
      • Cruz A.C.L.
      • Colella M.P.
      • De Paula E.V.
      • Annichinno-Bizzachi J.
      • Orsi F.A.
      Clinical course of primary immune thrombocytopenia with positive antiphospholipid antibodies.
      ]. 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 [
      • 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.
      ]. Outside this variant, thrombocytopenia is often mild (between 100 and 150 × 109/L). The prevalence of thrombocytopenia in APS was estimated between 20 and53% [
      • Artim-Esen B.
      • Diz-Küçükkaya R.
      • İnanç M.
      The significance and management of thrombocytopenia in antiphospholipid syndrome.
      ]. 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.
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