Cyclosporin A can achieve immune tolerance in a patient with severe haemophilia B and refractory inhibitors.
Immune tolerance induction (ITI) is described in a patient with severe haemophilia B complicated by the presence of an inhibitor. A number of ITI regimes were attempted without success and the patient suffered from frequent relapses and bleeding episodes. Successful ITI was achieved with the additional use of cyclosporin A. The patient developed nephrotic syndrome although had a negative Bethesda titre at this time. When cyclosporin A therapy was ceased, the inhibitor titre rose and the patient suffered again from bleeding episodes. Cyclosporin A was reintroduced at a lower dose. The patient has now received cyclosporin A for 10 years, during which time he has relapsed three times for short periods (2 weeks). He is also on prophylaxis with factor IX three times a week with preinfusion levels >1% and without bleeding.
Cross
D C A
DC
University of Edinburgh, Edinburgh, UK.
Van Der Berg
H M
HM
D019774:Blood Coagulation Factor Inhibitors / Q000097:blood; D001779:Blood Coagulation Factors / Q000627:therapeutic use; D002675:Child, Preschool; D016572:Cyclosporine / Q000009:adverse effects / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005164:Factor IX / Q000627:therapeutic use; D002836:Hemophilia B / Q000188:drug therapy* / Q000276:immunology; D006801:Humans; D007108:Immune Tolerance; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use*; D007518:Isoantibodies / Q000097:blood; D008297:Male; D009404:Nephrotic Syndrome / Q000276:immunology; D012008:Recurrence
D019774:Blood Coagulation Factor Inhibitors; D001779:Blood Coagulation Factors; D007166:Immunosuppressive Agents; D007518:Isoantibodies; D016572:Cyclosporine; D005164:Factor IX; C065655:anti-inhibitor coagulant complex