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Right Atrial Thrombosis in Antiphospholipid Syndrome with Secondary Immune Thrombocytopenia

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      Background Antiphospholipid syndrome (APS) is an acquired thrombophilia that can be associated with decreased platelet counts.

      Case A 67-year-old woman presented with thrombocytopenia and a symptomatic right atrial mass suspicious of cardiac myxoma. Prolongation of the activated partial thromboplastin time (aPTT) was caused by a strong lupus anticoagulant, and bone marrow cytology was consistent with accelerated platelet clearance. The patient underwent uneventful resection of the atrial tumor, which turned out to be a calcified fibrin-rich thrombus. Definitive APS was diagnosed and long-term anticoagulation recommended.

      Conclusion When evaluating patients with right atrial masses, findings of thrombocytopenia and/or aPTT prolongation should raise the suspicion of APS-associated thrombosis.

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      Most cited references 11

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      International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS).

      New clinical, laboratory and experimental insights, since the 1999 publication of the Sapporo preliminary classification criteria for antiphospholipid syndrome (APS), had been addressed at a workshop in Sydney, Australia, before the Eleventh International Congress on antiphospholipid antibodies. In this document, we appraise the existing evidence on clinical and laboratory features of APS addressed during the forum. Based on this, we propose amendments to the Sapporo criteria. We also provide definitions on features of APS that were not included in the updated criteria.
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        Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international working group.

        Diagnosis and management of immune thrombocytopenic purpura (ITP) remain largely dependent on clinical expertise and observations more than on evidence derived from clinical trials of high scientific quality. One major obstacle to the implementation of such studies and in producing reliable meta-analyses of existing data is a lack of consensus on standardized critical definitions, outcome criteria, and terminology. Moreover, the demand for comparative clinical trials has dramatically increased since the introduction of new classes of therapeutic agents, such as thrombopoietin receptor agonists, and innovative treatment modalities, such as anti-CD 20 antibodies. To overcome the present heterogeneity, an International Working Group of recognized expert clinicians convened a 2-day structured meeting (the Vicenza Consensus Conference) to define standard terminology and definitions for primary ITP and its different phases and criteria for the grading of severity, and clinically meaningful outcomes and response. These consensus criteria and definitions could be used by investigational clinical trials or cohort studies. Adoption of these recommendations would serve to improve communication among investigators, to enhance comparability among clinical trials, to facilitate meta-analyses and development of therapeutic guidelines, and to provide a standardized framework for regulatory agencies.
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          Evidence-based recommendations for the prevention and long-term management of thrombosis in antiphospholipid antibody-positive patients: report of a task force at the 13th International Congress on antiphospholipid antibodies.

           R Derksen,  R Brey,  V Pengo (2011)
          The antiphospholipid syndrome (APS) is defined by the presence of thrombosis and/or pregnancy morbidity in combination with the persistent presence of circulating antiphospholipid antibodies: lupus anticoagulant, anticardiolipin antibodies and/or anti-β2-glycoprotein I antibodies in medium to high titers. The management of thrombosis in patients with APS is a subject of controversy. This set of recommendations is the result of an effort to produce guidelines for therapy within a group of specialist physicians in Cardiology, Neurology, Hematology, Rheumatology and Internal Medicine, with a clinical and research focus on APS.

            Author and article information

            [1 ]Universitätsklinikum Hamburg-Eppendorf, II. Medizinische Klinik und Poliklinik, Onkologisches Zentrum, Hamburg, Germany
            [2 ]Universitätsklinikum Hamburg-Eppendorf, Klinik und Poliklinik für Herz- und Gefäßchirurgie, Universitäres Herzzentrum, Hamburg, Germany
            [3 ]Universitätsklinikum Hamburg-Eppendorf, Institut für Pathologie, Hamburg, Germany
            Author notes
            Address for correspondence Florian Langer, MD Universitätsklinikum Hamburg-Eppendorf II. Medizinische Klinik und Poliklinik, Onkologisches Zentrum, HamburgGermany f.langer@
            Thorac Cardiovasc Surg Rep
            Thorac Cardiovasc Surg Rep
            The Thoracic and Cardiovascular Surgeon Reports
            Georg Thieme Verlag KG (Stuttgart · New York )
            20 April 2015
            December 2015
            : 4
            : 1
            : 40-43
            4670312 10.1055/s-0035-1549841 140147crc
            © Thieme Medical Publishers


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