{
  "abstract": "Introduction Antiphospholipid syndrome (APS) is an autoimmune disease characterised by obstetric morbidity and recurrent venous and/or arterial thrombosis. It is frequently associated with systemic lupus erythematosus, and thrombocytopenia is a common manifestation. This study aimed to compare patients with APS, with and without thrombocytopenia, and to describe management and outcomes of severe thrombocytopenia.Methods We performed a retrospective single-centre study that included 432 patients with APS. Patients were divided into three groups according to the platelet count nadir: severe thrombocytopenia (< 50 G/L), moderate thrombocytopenia (between 50 and 130 G/L) and no thrombocytopenia (≥ 130 G/L).Results 142 patients developed thrombocytopenia during follow-up (32.9%): 57 (13%) had severe thrombocytopenia (< 50 G/L) and 85 (19.4%) had moderate thrombocytopenia (50–130 G/L). Patients with thrombocytopenia more frequently experienced thrombotic manifestations, including lower limb deep vein thrombosis (49% vs 37%, p=0.014), coronary thrombosis (15% vs 6.6%, p=0.006) and catastrophic antiphospholipid syndrome (CAPS) (27% vs 2.1%, p<0.001). Thrombocytopenia was more frequently associated with pre-eclampsia (16.8% vs 3.7%, p<0.001) and other APS manifestations, including skin (ulcer 4.9% vs 1.4%, p=0.046, cutaneous necrosis 7% vs 0.7%, p<0.001), cardiovascular (MINOCA 7% vs 0.7%, p<0.001, valvular disease 19.7% vs 9.3%, p=0.002), renal (18.3% vs 3.1%, p<0.001) and pulmonary (5.6% vs 1%, p=0.007) manifestations. Aetiologies of severe thrombocytopenia were mainly immune thrombocytopenia (ITP) and CAPS. In patients with severe thrombocytopenia, treatment followed the approach used for primary ITP, and anticoagulation was generally maintained even in the presence of bleeding episodes. Overall survival was poorer in patients with severe thrombocytopenia (p<0.0001).Conclusion Our results suggest that thrombocytopenia is associated with a more severe APS phenotype and increased mortality.",
  "authors": [
    {
      "affiliations": [
        "Assistance Publique-Hôpitaux de Paris (APHP), Groupe Hospitalier Pitié-Salpêtrière, French National Referral Center for Systemic Lupus Erythematosus, Antiphospholipid Antibody Syndrome and Other Autoimmune Disorders, Service de Médecine Interne 2, Institut E3M, Inserm UMRS, Centre d'Immunologie et des Maladies Infectieuses (CIMI-Paris), Sorbonne Université, Paris, France"
      ],
      "name": "Carine Schmidt"
    },
    {
      "affiliations": [
        "Assistance Publique-Hôpitaux de Paris (APHP), Groupe Hospitalier Pitié-Salpêtrière, French National Referral Center for Systemic Lupus Erythematosus, Antiphospholipid Antibody Syndrome and Other Autoimmune Disorders, Service de Médecine Interne 2, Institut E3M, Inserm UMRS, Centre d'Immunologie et des Maladies Infectieuses (CIMI-Paris), Sorbonne Université, Paris, France"
      ],
      "name": "Matthias Papo"
    },
    {
      "affiliations": [
        "Assistance Publique-Hôpitaux de Paris (APHP), Groupe Hospitalier Pitié-Salpêtrière, French National Referral Center for Systemic Lupus Erythematosus, Antiphospholipid Antibody Syndrome and Other Autoimmune Disorders, Service de Médecine Interne 2, Institut E3M, Inserm UMRS, Centre d'Immunologie et des Maladies Infectieuses (CIMI-Paris), Sorbonne Université, Paris, France"
      ],
      "name": "Dov Taïeb"
    },
    {
      "affiliations": [
        "Assistance Publique-Hôpitaux de Paris (APHP), Groupe Hospitalier Pitié-Salpêtrière, French National Referral Center for Systemic Lupus Erythematosus, Antiphospholipid Antibody Syndrome and Other Autoimmune Disorders, Service de Médecine Interne 2, Institut E3M, Inserm UMRS, Centre d'Immunologie et des Maladies Infectieuses (CIMI-Paris), Sorbonne Université, Paris, France"
      ],
      "name": "Raphaël Lhote"
    },
    {
      "affiliations": [
        "Assistance Publique-Hôpitaux de Paris (APHP), Groupe Hospitalier Pitié-Salpêtrière, French National Referral Center for Systemic Lupus Erythematosus, Antiphospholipid Antibody Syndrome and Other Autoimmune Disorders, Service de Médecine Interne 2, Institut E3M, Inserm UMRS, Centre d'Immunologie et des Maladies Infectieuses (CIMI-Paris), Sorbonne Université, Paris, France"
      ],
      "name": "Miguel Hié"
    },
    {
      "affiliations": [
        "Assistance Publique-Hôpitaux de Paris (APHP), Groupe Hospitalier Pitié-Salpêtrière, French National Referral Center for Systemic Lupus Erythematosus, Antiphospholipid Antibody Syndrome and Other Autoimmune Disorders, Service de Médecine Interne 2, Institut E3M, Inserm UMRS, Centre d'Immunologie et des Maladies Infectieuses (CIMI-Paris), Sorbonne Université, Paris, France"
      ],
      "name": "Micheline Pha"
    },
    {
      "affiliations": [
        "Assistance Publique-Hôpitaux de Paris (APHP), Groupe Hospitalier Pitié-Salpêtrière, French National Referral Center for Systemic Lupus Erythematosus, Antiphospholipid Antibody Syndrome and Other Autoimmune Disorders, Service de Médecine Interne 2, Institut E3M, Inserm UMRS, Centre d'Immunologie et des Maladies Infectieuses (CIMI-Paris), Sorbonne Université, Paris, France"
      ],
      "name": "Julien Haroche"
    },
    {
      "affiliations": [
        "Assistance Publique-Hôpitaux de Paris (APHP), Groupe Hospitalier Pitié-Salpêtrière, French National Referral Center for Systemic Lupus Erythematosus, Antiphospholipid Antibody Syndrome and Other Autoimmune Disorders, Service de Médecine Interne 2, Institut E3M, Inserm UMRS, Centre d'Immunologie et des Maladies Infectieuses (CIMI-Paris), Sorbonne Université, Paris, France"
      ],
      "name": "Fleur Cohen-Aubart"
    },
    {
      "affiliations": [
        "Assistance Publique-Hôpitaux de Paris (APHP), Groupe Hospitalier Pitié-Salpêtrière, French National Referral Center for Systemic Lupus Erythematosus, Antiphospholipid Antibody Syndrome and Other Autoimmune Disorders, Service de Médecine Interne 2, Institut E3M, Inserm UMRS, Centre d'Immunologie et des Maladies Infectieuses (CIMI-Paris), Sorbonne Université, Paris, France"
      ],
      "name": "Pierre-Louis Cariou"
    },
    {
      "affiliations": [
        "Assistance Publique-Hôpitaux de Paris (APHP), Groupe Hospitalier Pitié-Salpêtrière, French National Referral Center for Systemic Lupus Erythematosus, Antiphospholipid Antibody Syndrome and Other Autoimmune Disorders, Service de Médecine Interne 2, Institut E3M, Inserm UMRS, Centre d'Immunologie et des Maladies Infectieuses (CIMI-Paris), Sorbonne Université, Paris, France"
      ],
      "name": "Nassim Ait Abdallah"
    },
    {
      "affiliations": [
        "Assistance Publique-Hôpitaux de Paris (APHP), Groupe Hospitalier Pitié-Salpêtrière, French National Referral Center for Systemic Lupus Erythematosus, Antiphospholipid Antibody Syndrome and Other Autoimmune Disorders, Service de Médecine Interne 2, Institut E3M, Inserm UMRS, Centre d'Immunologie et des Maladies Infectieuses (CIMI-Paris), Sorbonne Université, Paris, France"
      ],
      "name": "Alexis Mathian"
    },
    {
      "affiliations": [
        "Assistance Publique-Hôpitaux de Paris (APHP), Groupe Hospitalier Pitié-Salpêtrière, French National Referral Center for Systemic Lupus Erythematosus, Antiphospholipid Antibody Syndrome and Other Autoimmune Disorders, Service de Médecine Interne 2, Institut E3M, Inserm UMRS, Centre d'Immunologie et des Maladies Infectieuses (CIMI-Paris), Sorbonne Université, Paris, France"
      ],
      "name": "Quentin Moyon"
    },
    {
      "affiliations": [
        "Assistance Publique-Hôpitaux de Paris (APHP), Groupe Hospitalier Pitié-Salpêtrière, French National Referral Center for Systemic Lupus Erythematosus, Antiphospholipid Antibody Syndrome and Other Autoimmune Disorders, Service de Médecine Interne 2, Institut E3M, Inserm UMRS, Centre d'Immunologie et des Maladies Infectieuses (CIMI-Paris), Sorbonne Université, Paris, France"
      ],
      "name": "Zahir Amoura"
    }
  ],
  "title": "Severe thrombocytopenia in antiphospholipid syndrome: a retrospective study of 432 patients",
  "uid": "06513a64-76ce-5d20-89bd-38b885409f5c"
}