refludan and Antiphospholipid-Syndrome

Topics: Adolescent; Antibodies, Antiphospholipid; Anticoagulants; Antiphospholipid Syndrome; Antithrombins; beta 2-Glycoprotein I; Diagnosis, Differential; Disease Progression; Dose-Response Relationship, Drug; Down Syndrome; Enoxaparin; Female; Hirudins; Humans; Immunoglobulin M; Recombinant Proteins; Renal Veins; Thrombophlebitis; Thrombosis; Vena Cava, Inferior

Topics: Anticoagulants; Antiphospholipid Syndrome; Cerebral Hemorrhage; Drug Administration Schedule; Half-Life; Heparin; Hirudins; Humans; Lupus Erythematosus, Systemic; Male; Middle Aged; Recombinant Proteins; Renal Insufficiency; Thrombocytopenia; Treatment Outcome

Treatment of patients with heparin-induced thrombocytopenia type II (HIT II) and thrombosis in some cases that represents a clinical challenge, which, if unrecognized, may lead to treatment delay or disease progression with potentially lethal outcome. We present a case of a 19-year-old patient with antiphospholipid syndrome, factor V (FV) Leiden mutation in heterozygous state, and venous thromboembolism. The patient was subjected to intravenous infusions of unfractionated heparin (UFH), and 16 days after the beginning of the treatment, his condition worsened, with thrombocytopenia and extension of thrombosis. Whereas the patient had a high clinical score for HIT II, functional and antigenic assays for the presence of HIT antibodies were negative. After repeated negative functional and antigenic assays, pseudo-HIT was suspected and nadroparin was introduced, which resulted in further worsening of the clinical presentation. Disease remission, along with complete normalization of platelet count, was finally accomplished with the introduction of lepirudin. The presence of multiple comorbid states, such as antiphospholipid syndrome, can potentially make laboratory confirmation of disease more difficult in patients with HIT II. In our opinion, it is of great importance that HIT II diagnosis be primarily clinical and that laboratory test results are carefully interpreted, especially when HIT is indicated by high clinical score values.

Topics: Administration, Oral; Adult; Antibodies; Anticoagulants; Antiphospholipid Syndrome; Diagnosis, Differential; Factor V; Heparin; Hirudins; Humans; Infusions, Intravenous; Male; Mutation; Nadroparin; Platelet Count; Recombinant Proteins; Thrombocytopenia; Venous Thromboembolism

To report a case of a patient with antiphospholipid antibody syndrome and multiple thromboses who developed heparin-induced thrombocytopenia (HIT) and subsequent international normalized ratio (INR) prolongation possibly due to antiphospholipid antibodies.. A 56-year-old white woman with a history of antiphospholipid antibody syndrome and thrombosis taking chronic warfarin was admitted for gastrointestinal concerns and found to have an INR >14. Warfarin was discontinued, vitamin K was administered, and a heparin infusion was initiated. Over the next 2 days, thrombocytopenia, hypotension, tachycardia, hyponatremia, and progressive abdominal pain developed. Upon transfer to a tertiary care center, HIT was diagnosed, and a lepirudin infusion was initiated. Subsequently, a sudden elevation of the INR occurred (>14) with low prothrombin (factor II) activity. After INR values declined to 2-3, warfarin was reinitiated with dosing adjusted using factor X and II activity levels. Clotting factors II and X activities were measured to monitor long-term warfarin therapy, with no evidence of complications after 7 months.. Typically, the INR is used to assess the intensity of anticoagulation. The INR value represents the reduction of clotting factors II, VII, and X. In rare circumstances, an independent inhibitor or interfering substance can interfere with the process of measuring the INR. In such situations, an alternative approach can be direct measurement of clotting factor concentrations.. Factor II and/or factor X activity levels provided an alternative means for measuring the anticoagulant effects of warfarin in the presence of a significant inhibitor (antiphospholipid antibodies) that biased the INR measurements.

Topics: Anticoagulants; Antiphospholipid Syndrome; Factor VII; Factor X; Female; Hirudins; Humans; International Normalized Ratio; Middle Aged; Platelet Count; Prothrombin; Recombinant Proteins; Thrombocytopenia; Warfarin

A patient with antiphospholipid antibody syndrome (APS) and a history of heparin-induced thrombocytopenia required lepirudin therapy. The patient had an abnormal baseline activated partial thromboplastin time (aPTT), complicating management of his therapy. We investigated whether an alternative monitoring system, using a dry reagent technology [Thrombolytic Assessment System (TAS)], could be used to monitor the patient's whole blood ecarin clot time (ECT) and aPTT. Baseline values for the ECT and aPTT were normal with this system. During a continuous infusion of lepirudin, the patient's whole blood ECT was maintained between a desired range of 150-200 s for 73% of the time. Similarly, his whole blood aPTT was maintained between 60 and 80 s for 80% of the time. In contrast, the patient's plasma-based aPTT by standard methods was consistently > 150 s. The patient underwent surgical procedures without complications. To further investigate the finding that the patient's antibody did not affect the aPTT with this system, we performed the ECT and the aPTT assays on the TAS Analyzer with plasma samples from 10 patients with APS and abnormal aPTTs. All 10 samples had plasma ECT values within the normal range. Four patients had normalization of the aPTT, suggesting that a subset of patients with APS may benefit from the TAS aPTT assay when monitoring heparin or other anticoagulation therapy.

Topics: Anticoagulants; Antiphospholipid Syndrome; Drug Monitoring; Endopeptidases; Heparin; Hirudins; Humans; Male; Middle Aged; Partial Thromboplastin Time; Recombinant Proteins; Thrombocytopenia; Whole Blood Coagulation Time