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Task Force Report on Non-criteria Manifestations: Thrombocytopenia

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Antiphospholipid Syndrome

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Abstract

Thrombotic manifestations, particularly when recurrent, are the antiphospholipid syndrome’s (APS) seal of identity. However, in a recent analysis comprising over 2,900 APS patients, the frequency of thrombocytopenia ranged from 30% to 46%. Accumulated data have also shown that between 10–15% of patients with immune thrombocytopenic purpura (ITP) may develop thrombosis depending upon their antiphospholipid antibody (aPL) profile. These statements could mean that these two apparent different settings are one and the same: “ITP” patients with positive aPL and patients with thrombocytopenia as their only APS clinical manifestation. What appears to define these “two” settings is the presence of aPL with lupus anticoagulant activity. Regardless of this evidence, according to the APS Classification criteria, an aPL-positive patient with thrombocytopenia but without a history of thrombosis or pregnancy morbidity is not classified as having APS. Herein, we provide relevant information regarding the pathogenic and clinical similarities and differences of platelet destruction seen in APS and ITP. Finally, we review the current therapeutic decisions in aPL-associated thrombocytopenia based on expert opinion and international consensus report for the management of ITP.

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Acknowledgments

The members of the “Task Force on Catastrophic Antiphospholipid Syndrome (APS) and Noncriteria APS Manifestations” that contributed to the discussions that are presented in this chapter are as follows:

Ricard Cervera and Gerard Espinosa (Department of Autoimmune Diseases, Hospital Clinic, Barcelona, Catalonia, Spain), Maria G. Tektonidou (First Department of Internal Medicine, Medical School, National University of Athens, Athens, Greece), Antonio R. Cabral (Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico), Emilio B. González (Division of Rheumatology, Department of Medicine, The University of Texas Medical Branch, Galveston, Texas, USA), Doruk Erkan (The Barbara Volcker Center for Women and Rheumatic Disease, Hospital for Special Surgery, Weill Medical College of Cornell University, New York, NY, USA), Smita Vadya (Department of Pathology, University of Texas Medical Branch, Galveston, Texas, USA), Horacio E. Adrogué (The Methodist Hospital Transplant Center, Fannin, Houston, Texas, USA), Michal Solomon (Department of Dermatology, Chaim Sheba Medical Center, Tel Hashomer, Israel), Gisele Zandman-Goddard (Department of Medicine C, Wolfson Medical Center, Tel Hashomer, Israel), and Yehuda Shoenfeld (Zublodovitz Center for Autoimmune Diseases and Department of Medicine B, Chaim Sheba Medical Center, Tel Hashomer, Israel).

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Authors and Affiliations

  1. Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga 15, Mexico City, DF, 014000, Mexico

    Gabriela Hernández-Molina MD & Antonio R. Cabral MD

  2. Department of Hematology and Oncology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, DF, Mexico

    Xavier López-Karpovitch MD

  3. Department of Medicine and Rheumatology, The University of Texas Medical Branch, Galveston, TX, USA

    Emilio B. González MD

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  1. Gabriela Hernández-Molina MD
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  2. Xavier López-Karpovitch MD
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  3. Emilio B. González MD
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  4. Antonio R. Cabral MD
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Correspondence to Antonio R. Cabral MD .

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Editors and Affiliations

  1. The Barbara Volcker Center for Women and Rheumatic Diseases, Hospital for Special Surgery, New York, NY, USA

    Doruk Erkan

  2. University of Texas Medical Branch, 301 University Boulevard, Galveston, 77555-0883, Texas, USA

    Silvia S. Pierangeli

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Hernández-Molina, G., López-Karpovitch, X., González, E.B., Cabral, A.R. (2012). Task Force Report on Non-criteria Manifestations: Thrombocytopenia. In: Erkan, D., Pierangeli, S. (eds) Antiphospholipid Syndrome. Springer, Boston, MA. https://doi.org/10.1007/978-1-4614-3194-7_13

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  • DOI: https://doi.org/10.1007/978-1-4614-3194-7_13

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