Immune tolerance in a haemophilia A patient with high inhibitor using locally prepared lyophilized cryoprecipitate

Abstract

The immune tolerance in a 12-year-old haemophilia A patient was carried out at the Faculty of Medicine, Ramathibodi Hospital, Bangkok in 1998. His inhibitor titres ranged from 10 to 3450 Bethesda units (BU). He suffered from serious bleeding episodes requiring prolonged hospitalization and the disarticulation of the left knee joint. After obtaining informed consent, locally prepared lyophilized cryoprecipitate (LC), heat treated at 60 degrees C for 25 h, was given in a dose of 13 units kg-1 body weight of factor VIII three times per week. His inhibitor was increased from 15 to 580 BU within the first 4 weeks of immune tolerance. Finally, it was decreased to 40 BU in the 36th week. The only adverse effect was seroconversion of anti-hepatitis C virus after receiving 108 bottles of LC for 36 weeks. In conclusion, the locally prepared LC was able to control the bleeding episodes in a haemophilia A patient with high inhibitor. To our knowledge, this is the first reported case in Thailand.

Comprehensive Care and Delivery of Care in Hemophilia: The Developing World

“…The optimal dose remains to be defined and varies. In Turkey, four out of seven patients underwent successful immune tolerance therapy with 25 IU/kg factor VIII three times weekly over 1-4 months [33,34]. Though stopped prematurely, the International ITI study, a multicenter prospective randomized study looking at high dose (200 IU/kg/ day) vs low dose (50 IU/kg/day 3 times/week) in patients with high-titer FVIII inhibitor did not find any difference in overall success of the two protocols [31], though breakthrough bleeds were higher in the lower dose protocols.…”

Management of haemophilia patients in Thailand

“…Lyophilized cryoprecipitate was first used for immune tolerance induction (ITI) in a 12-year-old boy with a high inhibitor titre of 3450 Bethesda units (BU) in 1998. The patient could attend a regular school and the parents could go to work during a 1-year period of ITI [10].…”

Hemophilia Treatment in Developing Countries: Products and Protocols

“…Immune tolerance induction is occasionally attempted with locally prepared cryoprecipitate. 4 Adjuvant Therapy DDAVP is available only in intravenous form and is used for patients with mild disease. Tranexamic acid is used for dental procedures and mucosal bleeds.…”

“…E-mail: aloks@cmcvellore.ac.in. 1 Professor, Christian Medical College, Vellore, India; 2 Korean Hemophilia Foundation, Seoul, Korea; 3 National Blood Centre, Kuala Lumpur, Malaysia; 4 Ramathibodi Hospital, Bangkok, Thailand; 5 Centro Nacional de Hemofilia, Caracas, Venezuela; 6 Hematological Research Institute, Buenos Aires, Argentina; 7 Iranian Hemophilia Society, Teheran, Iran. Copyright # 2005 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA.…”