Document Detail

Frequency of inhibitor development in severe haemophilia A children treated with cryoprecipitate and low-dose immune tolerance induction.
MedLine Citation:
PMID:  11122388     Owner:  NLM     Status:  MEDLINE    
The frequency of factor VIII inhibitor development was evaluated in a hundred severe haemophilia A patients < 18 years of age (mean 10.4 +/- 5.1 years); 25 were previously untreated patients (PUPs), with a mean age of 11.2 +/- 2.9 months. All were followed up for 3 years from December 1996. Immune tolerance (IT) was induced with low-dose factor VIII (FVIII); 25-50 IU kg(-1) every other day for the 10 haemophiliacs who developed persistent inhibitors. The incidence of inhibitors for PUPs was 3/25 (12%; 95% confidence interval [CI], 0. 7-24.7%) and were detected after 4, 15 and 20 exposure days (mean 13 +/- 8.2 days; 95% CI, 3.7-22.2%). Children with maximum inhibitor levels of > 40 Bethesda units (BU) per mL (n=4) received IT therapy as 25 U kg(-1) FVIII in the form of cryoprecipitate every other day for 1-4 months (mean 2.4 +/- 1.6 months; 95% CI, 0.8-3.9%), which was successful in all of them. FVIII (50 U kg(-1)) was given every other day for six patients with maximum inhibitor level > 40 BU mL(-1) for 3-9 months (mean 5.4 +/- 3.2 months; 95% CI, 2.9 -7.9%) with success in 4/6 (66.6%; 95% CI, 28.8-104.3%). Patients who showed a good IT response had an inhibitor level < or = 30 BU mL(-1), were < or = 9 years of age at inhibitor development with few exposure days to FVIII and had an early immune tolerance. In conclusion, inhibitor development in severe haemophilia A children exclusively treated with cryoprecipitate is low. Early low-dose IT induction for high responders may be achieved successfully if inhibitor level is < or = 50 BU mL(-1).
M S ElAlfy; M S El Alfy; A A Tantawy; M H Ahmed; I A Abdin
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Haemophilia : the official journal of the World Federation of Hemophilia     Volume:  6     ISSN:  1351-8216     ISO Abbreviation:  Haemophilia     Publication Date:  2000 Nov 
Date Detail:
Created Date:  2001-02-02     Completed Date:  2001-02-02     Revised Date:  2011-11-03    
Medline Journal Info:
Nlm Unique ID:  9442916     Medline TA:  Haemophilia     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  635-8     Citation Subset:  IM    
Department of Paediatric Haematology, Ain Shams University, Cairo, Egypt.
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MeSH Terms
Antibodies / blood,  immunology*
Child, Preschool
Factor VIII / immunology*,  isolation & purification,  therapeutic use*
Hemophilia A / blood,  drug therapy*,  immunology*
Immune Tolerance
Reg. No./Substance:
0/Antibodies; 9001-27-8/Factor VIII
Erratum In:
Haemophilia. 2011 Nov;17(16):979
Note: El Alfy, M S [corrected to ElAlfy, M S]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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