1/11. Characterization of IgG monoclonal anti-cardiolipin/anti-beta2GP1 antibodies from two patients with antiphospholipid syndrome reveals three species of antibodies.Antiphospholipid antibodies (aPL), including antibodies detected in anti-cardiolipin (aCL) enzyme-linked immunosorbent assays and in lupus anticoagulant (LA) tests, are strongly associated with recurrent thrombosis and recurrent fetal loss, i.e. the antiphospholipid syndrome (APS). Although recent studies suggest that most APS-associated aCL are directed against the phospholipid (PL)-binding plasma protein beta2-glycoprotein 1 (beta2GP1), the precise nature of aCL binding specificities remains controversial. To address the issue of aCL specificity we generated five new monoclonal IgG aCL from two patients with APS. Characterization of these five aCL, as well as two previously published IgG aCL, revealed three patterns of reactivity: (1) four antibodies reacted strongly with human beta2GP1-cardiolipin (CL) complexes and weakly with human beta2GP1 alone; (2) two antibodies recognized bovine beta2GP1, but not human beta2GP1; (3) one antibody reacted with complexes of human beta2GP1 and CL, but not with human beta2GP1 alone. Only one monoclonal displayed weak LA activity. These patient-derived IgG monoclonal antibodies, and additional ones to be generated, may help define varying species of antibodies detected in aCL assays and identify the specific antibodies that may be pathogenic.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/11. Catastrophic antiphospholipid syndrome triggered by trauma.We describe the first case of catastrophic antiphospholipid syndrome triggered by trauma. In contrast to reports that emphasize the devastating nature of the syndrome, our patient's course is less dramatic and more elusive. A possible pathophysiological explanation to the association of antiphospholipid syndrome and trauma is discussed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/11. Therapeutic plasma exchange for the acute management of the catastrophic antiphospholipid syndrome: beta(2)-glycoprotein I antibodies as a marker of response to therapy.We describe two patients with the catastrophic antiphospholipid syndrome associated with elevation of beta(2)-glycoprotein I antibodies and fulminant thrombotic diatheses. Both patients were treated with therapeutic plasma exchange (TPE), which resulted in a marked decrease in antibody titer accompanied by an improved clinical outcome in one patient (IgG antibody). In the second patient, the outcome was poor despite TPE (IgA antibody). There were no significant complications of TPE in either case. Because of the fulminant nature of the catastrophic antiphospholipid syndrome, we conclude that a trial of TPE is warranted for the acute management. Further studies are needed to clarify which patients may benefit from this treatment.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/11. Asymptomatic intracardiac thrombi and primary antiphospholipid syndrome.Intracardiac thrombi have rarely been reported in patients with the antiphospholipid syndrome. We describe a new case revealed by systematic echocardiography in an asymptomatic woman who consulted for mild thrombocytopenia. Our case is characterized by a past history of migraine, fetal loss and psychiatric disturbances and by a high level of antiphospholipid antibodies (anticardiolipin, anti-beta(2)-glycoprotein I and lupus anticoagulant). Echography, in the absence of any cardiovascular symptom, showed bulky right intra-atrial thrombi requiring surgical excision. Histopathological analysis revealed the fibrino-cruoric nature of the lesion without myxoma. This case shows that, when faced with a mild thrombocytopenia associated with antiphospholipid antibodies, echocardiography may reveal a life-threatening and completely asymptomatic intracardiac thrombosis.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/11. Fatal tumor thrombosis due to an inferior vena cava leiomyosarcoma in a patient with antiphospholipid antibody syndrome.We describe a patient with antiphospholipid antibody syndrome (APS) who died because of relentless inferior vena cava (IVC) tumor thrombosis due to an unsuspected leiomyosarcoma. Laboratory confirmation for APS was provided by functional identification of a lupus anticoagulant and anticardiolipin IgG and anti-beta2-glycoprotein I IgM antibodies. Although sensitive for detecting vascular obstruction, radiocontrast venography and magnetic resonance imaging and angiography detected the IVC thrombosis but failed to distinguish its malignant nature. Concomitant refractory thrombocytopenia prevented further invasive diagnostic and therapeutic maneuvers for progressive, severe IVC thrombosis unresponsive to aggressive treatment of APS. Deep venous thrombosis refractory to anticoagulant and immunomodulatory therapies in a patient with APS may be due to a concomitant underlying malignancy, such as a leiomyosarcoma, causing vascular obstruction.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/11. Isolated tricuspid valve vegetations and steno-insufficiency in primary antiphospholipid syndrome.Although cardiac valve disease is frequent in the antiphospholipid syndrome, isolated tricuspid valve pathology is uncommon and tricuspid valve steno-insufficiency with concomitant vegetations in association with primary antiphospholipid syndrome has very rarely been mentioned in the literature. We describe a 54-year-old Indian woman with newly-diagnosed antiphospholipid syndrome and isolated tricuspid valve disease, the nature of which was only discernible by transoesophageal echocardiography. This case report highlights the importance of being aware of antiphospholipid syndrome as the possible underlying diagnosis in isolated organic tricuspid valve disease and the need for transoesophageal echocardiography in such cases.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/11. Antiphospholipid antibody syndrome: recent findings on managing this challenging condition.patients with antiphospholipid syndrome can suffer recurrent thrombosis that is very difficult to manage. Recent research has demonstrated that high-intensity anticoagulation is not superior to standard therapy. Basic questions about the nature and treatment of this syndrome remain unanswered.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/11. Recurrent intracardiac mass in a pregnant woman with antiphospholipid syndrome.Clinical manifestations of the antiphospholipid syndrome result from thromboembolic phenomena that occur in all vascular territories. Cardiac manifestations frequently associated with this syndrome include valvular and myocardial lesions. We present a case report of primary antiphospholipid syndrome in a pregnant young woman with a right atrial mass detected by echocardiography. Its morphological characteristics presented problems in establishing differential diagnosis within atrial thrombus and atrial myxoma. This case was complicated by pulmonary embolism and recurrence of the mass within four months. Cardiac surgery was performed and two masses excised. Histopathological studies showed them to be thrombotic in nature.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/11. Antiphospholipid antibody syndrome associated with ovarian cancer. A new paraneoplastic syndrome?We describe a 41-year-old woman, in whom antiphospholipid antibody syndrome (APS) occurred at presentation, before the detection of an ovarian endometrial adenocarcinoma. This syndrome was characterized by widespread and worsening thromboembolism and it did not respond to conventional anticoagulant treatment. The paraneoplastic nature of this APS was strongly suggested by the disappearance of both thromboembolism and antiphospholipid antibodies only after surgical removal of the cancer.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
10/11. A monoclonal IgG anticardiolipin antibody from a patient with the antiphospholipid syndrome is thrombogenic in mice.Antiphospholipid antibodies, including anticardiolipin antibodies (ACA), are strongly associated with recurrent thrombosis in patients with the antiphospholipid syndrome (APS). To date, reports about the binding specificities of ACA and their role(s) in causing and/or sustaining thrombosis in APS are conflicting and controversial. The plasmas of patients with APS, usually containing a mixture of autoantibodies, vary in binding specificity for different phospholipids/cofactors and vary in in vitro lupus anticoagulant activity. Although in vivo assays that allow assessment of the pathogenic procoagulant activity of patient autoantibodies have recently been developed, the complex nature of the mixed species prevented determination of the particular species responsible for in vivo thrombosis. We have generated two human IgG monoclonal ACA from an APS patient with recurrent thrombosis. Both bound to cardiolipin in the presence of 10% bovine serum, but not in its absence, and both were reactive against phosphatidic acid, but were nonreactive against purified human beta-2 glycoprotein 1, dna, heparan sulfate, or four other test antigens. Both monoclonal autoantibodies lacked lupus anticoagulant activity and did not inhibit prothrombinase activity. Remarkably, one of the monoclonal antibodies has thrombogenic properties when tested in an in vivo mouse model. This finding provides the first direct evidence that a particular antiphospholipid antibody specificity may contribute to in vivo thrombosis.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
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