sepharose and Hemophilia-B

A 37-yr-old hemophiliac with factor IX antibody in high titer required operation for a pseudotumor growing invasively into the bone in his left elbow. Exchange plasma-pheresis was not feasible, mainly because of possible stimulation of factor IX inhibitor. In addition, the patient had antibodies against tissue antigens. The factor IX antibodies were of IgG type and adsorbable to protein-A-agarose. The antibodies were removed by extracorporeal adsorption of the plasma (separated intermittently in a cell centrifuge) to sterile protein-A-Sepharose in columns, operated by a computer. The treated plasma was retransfused. In this manner, a total of 6000 ml of plasma was depleted of antibodies, and the antibody titer, as well as the total immunoglobulin content, decreased to one-fifth of the original values. The remaining antibodies were neutralized by infusion of factor IX concentrate. Conventional substitution therapy, in combination with immunosuppression, was then possible. The operation was performed without complications. No signs of hemolysis, complement activation, or activation of the coagulation system occurred. In principle, the same form of treatment can be used in patients with other forms of antibodies.

Topics: Absorption; Adult; Antibodies; Blood Coagulation Tests; Bone Neoplasms; Complement System Proteins; Elbow Joint; Factor IX; Hemophilia B; Humans; Male; Plasmapheresis; Sepharose

An agarose plate method for detecting human alloantibodies (inhibitors) to coagulant factor IX has been developed. The assay is based on the ability of such antisera to inhibit the coagulation of a mixture of haemophilia B plasma, normal plasma and platelet subsitute in an agarose matrix. The agarose plate method was also adopted to measure levels of FIX antigen (IX:Ag) in plasma. Using this technique, 12 of 17 obligate carriers of haemophilia B demonstrated reduced levels of IX:Ag. Three of the five carriers with normal IX:Ag levels were members of kindred in which affected individuals had normal or near normal levels of IX:Ag.

Topics: Antigens; Blood Coagulation Tests; Factor IX; Female; Genetic Carrier Screening; Hemophilia B; Humans; Isoantibodies; Male; Sepharose

Topics: Antigens; Chromatography, Gel; Citrates; Factor VIII; Hemophilia B; Humans; Molecular Weight; Plasma; Ristocetin; Sepharose