1/401. Lupus anticoagulant syndrome: case report.A twenty seven year old female was referred to our department with deep vein thrombosis, abnormal activated partial thromboplastin time (aPTT) ratio 1:60 and prothrombin time (PT) INR of 3:11. She had history of loss of pregnancies previously. Coagulation tests with pooled normal fresh plasma did not correct a PTT because of a coagulation inhibitor and only partially corrected PT. kaolin clotting time (KCT) of patient plasma (PP) and a mixture of PP/normal plasma (NP) detected the lupus anticoagulant (LA). Venereal disease Laboratory (VDRL) test on the patient's serum was positive with low titre 1:8 while treponema pallidum haemaglutination test (TPHA) was negative. Anticardiolipin antibodies IgG were raised while IgM levels were within normal levels. This was a case of lupus anticoagulant syndrome. The patient was treated with unfractionated heparin and warfarin and later started on salicylates and prednisone.- - - - - - - - - - ranking = 1keywords = thrombosis (Clic here for more details about this article) |
2/401. factor v Leiden and antibodies against phospholipids and protein S in a young woman with recurrent thromboses and abortion.We describe the case of a 39-year-old woman who suffered two iliofemoral venous thromboses, a cerebral ischemic infarct and recurrent fetal loss. Initial studies showed high levels of antiphospholipid antibodies (APAs) and a moderate thrombocytopenia. After her second miscarriage, laboratory diagnosis revealed that the woman was heterozygous for the factor v Leiden mutation and had a functional protein s deficiency as well as anti-protein S and anti-beta 2-glycoprotein i antibodies. The impairment of the protein c pathway at various points could well explain the recurrent thromboses in the patient and supports the role of a disturbed protein c system in the pathophysiology of thrombosis in patients with APAs.- - - - - - - - - - ranking = 1.001642397241keywords = thrombosis, cerebral (Clic here for more details about this article) |
3/401. Clinical therapeutic conference: recurrent venous thrombotic and thromboembolic disease.Recurrent venous thrombotic and thromboembolic disease, once thought to be an uncommon entity, is increasingly being recognized. Etiologies of recurrent deep venous thrombosis usually include elements of Virchow's triad. Venous stasis (e.g., immobilization, congestive heart failure, acute myocardial infarction, obesity), hypercoagulability (e.g., malignancy, inflammatory bowel disease, hyperhomocysteinemia, protein c resistance, antithrombin III, protein c or S deficiency) and endothelial trauma (e.g., surgical trauma, venous trauma, in-dwelling venous instrumentation) are risk factors. diagnosis is dependent on objective testing, including venography duplex Doppler (color) ultrasonography and impedance plethysmography. Treatment is usually started with heparin or low-molecular-weight heparin and advanced to warfarin (adjusted to international normalized ratio). Prophylaxis may continue using low-molecular-weight heparin, warfarin, venacaval interruption (Greenfield filter), or concomitant use of the platelet-active agent indobufen and graduated compression stockings.- - - - - - - - - - ranking = 1keywords = thrombosis (Clic here for more details about this article) |
4/401. Cryosupernatant plasma exchange in the treatment of antiphospholipid antibody syndrome with lupus nephritis.We report a case of a 22-year-old female with antiphospholipid antibody syndrome (APS) associated with systemic lupus erythematosus in whom cryosupernatant plasma exchange was effective and improved both the refractory venous thrombosis in her legs and relapsing thrombocytopenia. A renal biopsy specimen showed not only features of active lupus nephritis but also renal arteriolar thrombosis which is considered to be a type of thrombotic microangiopathy (TMA). Because a pathological role of unusually large von willebrand factor (vWF) multimers has been reported in patients with TMA including thrombotic thrombocytopenic purpura, plasma exchange using replacement with cryosupernatant, which is free of unusually large vWF multimers, is likely to be an option of treatment modality for patients with refractory and chronic relapsing APS manifesting TMA.- - - - - - - - - - ranking = 2keywords = thrombosis (Clic here for more details about this article) |
5/401. Diffuse alveolar hemorrhage in the antiphospholipid syndrome: spectrum of disease and treatment.OBJECTIVE: To describe clinical manifestations, laboratory findings, and treatment of patients with the antiphospholipid syndrome (APS) who develop diffuse alveolar hemorrhage. methods: Diffuse alveolar hemorrhage is an occasionally reported manifestation of the APS. The diagnosis, however, may be overlooked or manifestations attributed to another disease process. Seven episodes in 5 patients with primary APS were identified and retrospectively reviewed for presenting symptoms and signs, laboratory findings, and response to treatment. RESULTS: The severity of the condition varies, and diffuse alveolar hemorrhage may be the initial manifestation of APS. patients may present with symptoms ranging from cough, dyspnea, and fever with or without hemoptysis, to symptoms of acute respiratory failure. Hypoxemia and anemia are usually present. Other causes need to be excluded. bronchoscopy and bronchoalveolar lavage with or without biopsy often aid in confirming the diagnosis. The pathologic abnormality appears to be microvascular thrombosis with or without capillaritis. Treatment with corticosteroids usually leads to marked improvement. CONCLUSION: patients with APS may present with diffuse alveolar hemorrhage resulting in mild to life threatening symptoms. Prompt and thorough evaluation to confirm the diagnosis and treatment with corticosteroids usually leads to rapid improvement. The clinical setting will dictate whether other therapies such as immunosuppressive agents or intravenous immunoglobulin are required.- - - - - - - - - - ranking = 1keywords = thrombosis (Clic here for more details about this article) |
6/401. 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 = thrombosis (Clic here for more details about this article) |
7/401. Primary antiphospholipid syndrome: a cause of catastrophic shunt thrombosis in the newborn.This is a unique report of systemic-to-pulmonary artery shunt thromboses secondary to primary antiphospholipid syndrome and antithrombin iii deficiency in a neonate with cyanotic congenital heart disease. This infant with tricuspid atresia experienced thromboses of two modified Blalock-Taussig shunts en route to a bidirectional cavo-pulmonary shunt and potential future Fontan operation. Chronic warfarin anticoagulation has prevented additional thrombo-embolic events.- - - - - - - - - - ranking = 4keywords = thrombosis (Clic here for more details about this article) |
8/401. Primary antiphospholipid syndrome and cerebral atrophy: a rare association?Neurologic complications are common in patients with antiphospholipid syndrome. In this article, we report the case of a young woman with neurologic disorders, a history of hypertension and transient ischemic attacks, and cerebral atrophy associated with primary antiphospholipid syndrome (PAPS). magnetic resonance imaging of the brain showed multiple ischemic lesions and remarkable atrophy of frontal and parietal lobes. Cerebral atrophy in patients with PAPS can be considered as a feature of this disease. The case is discussed on the basis of relevant past literature. Although there are few reports on neuroradiologic findings in patients with PAPS, cerebral atrophy has been described. Because PAPS is more frequently recognized today than in the past, this condition should be included in the differential diagnosis of cerebral atrophy, particularly in young patients.- - - - - - - - - - ranking = 0.011534027992214keywords = cerebral, brain (Clic here for more details about this article) |
9/401. Recurrent thrombo-embolic episodes: the association of cholangiocarcinoma with antiphospholipid syndrome.antiphospholipid syndrome is a disorder of recurrent vascular thrombosis, pregnancy loss and thrombocytopenia associated with persistently elevated levels of antiphospholipid antibodies. It was first described in a group of patients with systemic lupus erythematosus but has since been associated with a wide range of conditions, including other autoimmune disorders and malignancy. It can also occur in isolation, the so-called primary antiphospholipid syndrome. We describe an elderly woman with the antiphospholipid syndrome thought to be associated with a cholangiocarcinoma.- - - - - - - - - - ranking = 1keywords = thrombosis (Clic here for more details about this article) |
10/401. Two cases of autoimmune thrombocytopenic purpura associated with antiphospholipid antibodies.We report 2 cases of autoimmune thrombocytopenic purpura associated with antiphospholipid antibodies. In the first case the titer of antiphospholipid antibodies was neither related to disease activity nor influenced by immunosuppressive therapy. In the second, cerebral infarction occurred in spite of severe thrombocytopenia. Our results provide additional evidence that antiphospholipid antibodies are not involved in the pathogenesis of autoimmune thrombocytopenic purpura. Nevertheless, the frequency and clinical significance of this association would suggest that patients with autoimmune thrombocytopenic purpura should be tested for antiphospholipid antibodies, particularly before pregnancy or surgical procedures.- - - - - - - - - - ranking = 0.0016423972410095keywords = cerebral (Clic here for more details about this article) |
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