Immune tolerance in a haemophilia A patient with high inhibitor using locally prepared lyophilized cryoprecipitate.
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.
Chuansumrit
A
A
Department of Paediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand. raajs@mahidol.ac.th
Pakakasama
S
S
Kuhathong
K
K
Chantanakajornfung
A
A
Hathirat
P
P
D011232:Chemical Precipitation; D002648:Child; D015925:Cryopreservation; D005169:Factor VIII / Q000008:administration & dosage* / Q000009:adverse effects / Q000276:immunology; D005612:Freeze Drying; D006467:Hemophilia A / Q000188:drug therapy* / Q000276:immunology / Q000821:virology; D006470:Hemorrhage / Q000188:drug therapy; D006526:Hepatitis C / Q000209:etiology; D006801:Humans; D007108:Immune Tolerance / Q000187:drug effects*; D007518:Isoantibodies / Q000097:blood*; D008297:Male
D007518:Isoantibodies; D005169:Factor VIII