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Cyclosporin A can achieve immune tolerance in a patient with severe haemophilia B and refractory inhibitors
Author(s) -
CROSS D. C. A.,
VAN DEN BERG H. M.
Publication year - 2007
Publication title -
haemophilia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.213
H-Index - 92
eISSN - 1365-2516
pISSN - 1351-8216
DOI - 10.1111/j.1365-2516.2006.01411.x
Subject(s) - medicine , haemophilia , refractory (planetary science) , haemophilia b , haemophilia a , factor ix , titer , nephrotic syndrome , surgery , immune tolerance , gastroenterology , immune system , immunology , antibody , physics , astrobiology
Summary. 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.