Cryptic conspirators: a conversation about thrombocytopenia and antiphospholipid syndrome

Purpose of review Although antiphospholipid syndrome (APS) is best known for conveying increased risk of thrombotic events and pregnancy morbidity, thrombocytopenia is also recognized as a common association. In this review, we will explore the relationship between thrombocytopenia and APS, highlighting our evolving understanding – and persistent knowledge gaps – through clinically oriented questions and answers. Recent findings A history of thrombocytopenia likely portends a more severe APS phenotype (including increased risk of thrombosis). Although the pathophysiology underlying thrombocytopenia in APS has yet to be definitively revealed, mechanisms that play a role (at least in subsets of patients) include: immune thrombocytopenic purpura/ITP-like autoantibodies against platelet glycoproteins; antiphospholipid antibody (aPL)-mediated platelet activation and consumption; and potentially life threatening thrombotic microangiopathy. Although thrombocytopenia is often ‘mild’ in APS (and therefore, may not require specific therapy), there are causes of acute-onset thrombocytopenia that mandate emergent work-up and treatment. When APS-related thrombocytopenia does require therapy, the approach must be individualized (requiring an understanding of pathophysiology in the particular APS patient). For patients with ITP-like disease, rituximab is emerging as a popular approach to treatment; in contrast, there are hints that thrombopoietin mimetics may be...
Source: Current Opinion in Rheumatology - Category: Rheumatology Tags: CLINICAL THERAPEUTICS & HEMATOLOGIC COMPLICATIONS: Edited by W. Joseph McCune Source Type: research

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The objective of the present study was to review the pathophysiology of APS and its association with female infertility. A bibliographic review of articles of the past 20 yearswas performed at the PubMed, Scielo, and Bireme databases. Antiphospholipid antibody syndrome may be associated with primary infertility, interfering with endometrial decidualization and with decreased ovarian reserve. Antiphospholipid antibodies also have direct negative effects on placentation, when they bind to the trophoblast, reducing their capacity for invasion, and proinflammatory effects, such as complement activation and neutrophil recruitme...
Source: Revista Brasileira de Ginecologia e Obstetricia - Category: OBGYN Source Type: research
ConclusionBased on the patient ’s medical history and all the examination results, it was possible to make a diagnosis of primary APS and, starting from diagnosis of thrombocytopenia, we were allowed to conclude that all of manifestation were epi-phenomena of a unique clinical entity, rather than unrelated diseases. Though APS is one of the most common thrombocytophilias, unfortunately, it is not recognized often enough. The lack of prevention in undiagnosed patients may cause severe complications which can in turn result in the death of those patients.
Source: Autoimmunity Highlights - Category: Allergy & Immunology Source Type: research
Paola Adele Lonati1†, Mariangela Scavone2,3†, Maria Gerosa4, Maria Orietta Borghi1,4, Francesca Pregnolato1, Daniele Curreli1, Gianmarco Podda2,3, Eti Alessandra Femia2,3, Wilma Barcellini5, Marco Cattaneo2,3, Francesco Tedesco1 and Pier Luigi Meroni1* 1Immunorheumatology Research Laboratory, Istituto Auxologico Italiano, IRCCS, Milan, Italy2Unità di Medicina II, ASST Santi Paolo e Carlo, Milan, Italy3Dipartimento di Scienze Della Salute, University of Milan, Milan, Italy4Dipartimento di Scienze Cliniche e di Comunità, University of Milan, Milan, Italy5UOC Ematologia, Fondazione IRCCS Ca&#x...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
Authors: Darmawan G, Hamijoyo L, Oehadian A, Bandiara R, Amalia L Abstract A 38-year-old woman presented with general weakness and vaginal bleeding. One month prior, she had been diagnosed with Evans syndrome (haemolytic anemia with positive Coombs test and thrombocytopenia) and was given oral steroid as maintenance therapy. Her serology examination was negative for hepatitis B, hepatitis C, and human immunodeficiency virus (HIV). Her obstetrical history was marked by miscarriage in second pregnancy and preeclampsia in third pregnancy. She used hormonal contraceptives until 5 months prior to admission. On physical ...
Source: Acta medica Indonesiana - Category: Internal Medicine Tags: Acta Med Indones Source Type: research
We present a nulliparous pregnant woman with APS at the 30+6 week of gestation who has developed thrombocytopenia, intravascular hemolysis, elevated creatinine, proteinuria, and hematuria. Diagnoses: These featurs were compatible with the diagnosis of CAPS. Consensually, serum C3 protein levels were rapidly decreasing, reflecting complement consumption. Interventions: She was treated with eculizumab, a humanized monoclonal antibody against C5 that prevents the formation of the complement membrane attack complex. Outcomes: Laboratory parameters improved and the patient did not develop thrombosis or detectable organ...
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research
Abstract Antiphospholipid syndrome (APS) is an autoantibody-mediated acquired thrombophilia. It is characterized by the presence of antiphospholipid antibodies (APL) that are directed against phospholipid-binding plasma proteins, such as beta-2-glycoprotein I (b2GPI). Its main manifestations are recurrent vascular thromboses (so-called "thrombotic APS") and pregnancy complications ("obstetric APS"). According to the current consensus criteria, a persistently positive functional lupus anticoagulant (LA) assay and/or the presence of anti-b2GPI and/or anti-cardiolipin antibodies, together with cli...
Source: VASA. Zeitschrift fur Gefasskrankheiten. Journal for Vascular Diseases - Category: Surgery Authors: Tags: Vasa Source Type: research
Antiphospholipid Syndrome (APS) important in vascular medicine as well as obstetrics. In obstetrics, it is important because it can cause fetal loss, intrauterine growth retardation and severe preeclampsia. In vascular medicine it is important because it can cause thrombotic events which could be arterial, venous or microvascular [1]. It can also be accompanied by moderate thrombocytopenia [2]. Thrombotic events involving multiple organs may be termed catastrophic Antiphospholipid Syndrome [3]. APS is an autoimmune disease with antibodies directed against beta2 glycoprotein I. This leads to suppression of tissue fact...
Source: Cardiophile MD - Category: Cardiology Authors: Tags: General Cardiology anti beta2 glycoprotein I antibodies anti cardiolipin antibodies lupus anticoagulant Source Type: blogs
Summary Antiphospholipid syndrome (APS) is an autoimmune disease characterized by antiphospholipid antibodies (aPL) associated with thrombosis and/or pregnancy morbidity. Most APS events are directly related to thrombotic events, which may affect small, medium or large vessels. Other clinical features like thrombocytopenia, nephropathy, cardiac valve disease, cognitive dysfunction and skin ulcers (called non-criteria manifestations) add significant morbidity to this syndrome and represent clinical situations that are challenging. APS was initially described in patients with systemic lupus erythematosus (SLE) but it can occ...
Source: Revista da Associacao Medica Brasileira - Category: General Medicine Source Type: research
(APS) is an acquired autoimmune thrombophilia characterized by venous or arterial thrombosis, and/or pregnancy loss or complications in the presence of persistently positive antiphospholipid antibodies. Patients can also develop other organ involvement, referred to as non-criteria manifestations, including livedo reticularis, thrombocytopenia and nephropathy. Non-thrombotic inflammatory mechanisms are increasingly identified in the pathogenesis of APS, alongside a recognition that obstetric APS may be a specific subset of APS.
Source: Medicine - Category: Internal Medicine Authors: Tags: Other autoimmune disorders Source Type: research
Shilpa Sarvesh Urolagin, Deepthi Swaroop, Charu Agrawal, Pathik Dholakia, Menaka Basavanneppa KaralwadJournal of Indian Society of Periodontology 2016 20(5):561-564 Antiphospholipid antibody (APLA) syndrome is a noninflammatory autoimmune disease, with innumerable clinical manifestations ranging from recurrent thrombosis and pregnancy morbidity to valvular lesions, transverse myelitis, thrombocytopenia, and hemolytic anemia. APLAs in antiphospholipid syndrome (APS) are well-known risk factors for cerebrovascular accidents. Stroke is the most common manifestation of APS in the central nervous system. Gingival enlargement i...
Source: Journal of Indian Society of Periodontology - Category: Dentistry Authors: Source Type: research
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