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Antiphospholipid Antibody Syndrome

Circulating proteins that interfere with coagulation reactions can lead to serious bleeding diatheses. These anticoagulants are usually found to be IgG immunoglobulins and less often IgM or IgA antibodies that are directed against a specific coagulation factor antiphospholipid antibody syndrome, antiphospholipid syndrome. There is one so-called anticoagulant that prolongs clotting reactions in vitro, but actually leads to thrombosis rather than to bleeding in vivo, the Lupus Anticoagulant; it is the most common acquired "anticoagulant" observed. The

I. Antiphospholipid antibody syndrome (Lupus anticoagulant syndrome, APS)

This syndrome is defined by the presence of clinical criteria including 1) arterial or venous thrombosis, and/or 2) pregnancy morbidity that includes multiple miscarriages; and criteria including 1) the presence of an IgG or Ig M anticardiolipin antibody of medium or high titer, or 2) a lupus anticoagulant. One or both of the laboratory findings must be present on two or more occasions at least 6 weeks apart. Clinical findings may also include thrombocytopenia, autoimmune hemolytic anemia, livedo reticularis, cardiac valve disease (Libman-Sacks-like), and

Treatment of APES: Patients with APS and thrombosis require anticoagulation. They are treated with heparin for acute events, followed by warfarin to maintain the b at 3; low dose ASA is

 Thus, it appears that long-term anticoagulation is warranted in these patients who have a thrombotic event if the risk of bleeding is not judged to be too high in the individual patient. Patients with recurrent fetal loss benefit from administration of LMW heparin and low-dose ASA during their pregnancies, with an increase in

While factor VIII inhibitors occur in approximately 25% of hemophiliacs, their occurrence in non-hemophilic patients is rare. In the latter population, these inhibitors may occur spontaneously or in association with autoimmune diseases such as SLE and rheumatoid arthritis, pregnancy, or

Clinical manifestations. Patients usually present with ecchymoses and soft tissue bleeding in muscles, but occasionally joint, mucous membrane, and CNS bleeding may occur. Large intramuscular hematomas may appear at sites of inapparent injury on the extremities or in

Diagnosis of Factor VIII inhibitors. Laboratory evaluation shows a prolonged aPTT that does not correct by mixing with normal plasma. (A very low titer inhibitor may allow correction in the assay if measured at an immediate time point, but it will prolong the aPTT after incubation with normal plasma for 1-2 hours at 37EC.) A factor VIII assay indicates a low factor VIII level, and a factor VIII inhibitor assay using the Bethesda method shows the presence of the

Treatment of acquired inhibitors to factor VIII. Treatment of acute bleeding episodes requires careful nursing care with avoidance of trauma and invasive procedures such as IM injections. Specific coagulation factor therapy includes the use of porcine factor VIII, 50-100 U/kg q 8-12 hr (which usually cross-reacts much less with the antibody than human factor VIII), or, if the antibody titer is less than 5 Bethesda units, very high doses of human factor VIII by