Intensive exposure to factor VIII is a risk factor for inhibitor development in mild hemophilia A: Inhibitors in mild hemophilia A following continuous infusion of factor VIII

A. Sharathkumar; D. Lillicrap; V. S. Blanchette; M. Kern; J. Leggo; A. M. Stain; L. Brooker; M. D. Carcao

doi:10.1046/j.1538-7836.2003.00230.x

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Intensive exposure to factor VIII is a risk factor for inhibitor development in mild hemophilia A: Inhibitors in mild hemophilia A following continuous infusion of factor VIII

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Intensive exposure to factor VIII is a risk factor for inhibitor development in mild hemophilia A: Inhibitors in mild hemophilia A following continuous infusion of factor VIII

A. Sharathkumar, D. Lillicrap, V. S. Blanchette, M. Kern, J. Leggo, A. M. Stain, L. Brooker and M. D. Carcao

Journal of thrombosis and haemostasis, Vol.1(6), pp.1228-1236

06/2003

DOI: 10.1046/j.1538-7836.2003.00230.x

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Abstract

Background: Inhibitors are rare in boys with mild hemophilia A (MHA; factor (F)VIII:C >5%) but may arise following intense FVIII exposure, e.g. continuous infusion (CI). Objectives: To determine the impact of intenseFVIII exposure in inhibitor formation in MHA at our institution and to compare this with previous reports. Patients and methods: Were viewed FVIII exposure and inhibitor development in boys (ages 0-18 years) with MHA followed at our institution from 1996 to 2001 and conducted a Medline search (1966-2002) on the experience of inhibitor development following intensive/CI exposure to FVIII. Results: We identified 54 boys with MHA. Twenty-nine (54%) had been exposed to FVIII. Seven had received FVIII by CI. Four developed inhibitors; three high titer(at ages 10 years, 16 years and 17 years) and one low titer (at 1 month old).All four had received a CI of recombinant (r) FVIII of at least 6 days within 6 weeks of developing inhibitors. Baseline FVIII levels fell to <1% in all cases and the three with high-titer inhibitors developed severe bleeding. Immune tolerance therapy (ITT) was attempted in two boys and was successful in one. Our literature search identified 35 cases (only four children) with MHA developing inhibitors following intense FVIII exposure often in the context of surgery. Conclusions: The incidence of inhibitors in our MHA population was 7.4%. If expressed according to exposure the incidence was significantly higher: 14% (4/29) for any exposure to FVIII and 57% (4/7) for exposure by CI. A prospective study to address whether CI is associated with an increased incidence of inhibitor development in MHA is warranted. © 2003 International Society on Thrombosis and Haemostasis.

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Title: Subtitle: Intensive exposure to factor VIII is a risk factor for inhibitor development in mild hemophilia A: Inhibitors in mild hemophilia A following continuous infusion of factor VIII
Creators: A. Sharathkumar - Division of Haematology/Oncology, Department of Paediatrics and
D. Lillicrap - Queen's University
V. S. Blanchette - Division of Haematology/Oncology, Department of Paediatrics and
M. Kern - Division of Haematology/Oncology, Department of Paediatrics and
J. Leggo - Queen's University
A. M. Stain - University of Toronto
L. Brooker - Division of Haematology/Oncology, Department of Paediatrics and
M. D. Carcao - Division of Haematology/Oncology, Department of Paediatrics and
Resource Type: Journal article
Publication Details: Journal of thrombosis and haemostasis, Vol.1(6), pp.1228-1236
DOI: 10.1046/j.1538-7836.2003.00230.x
ISSN: 1538-7933
eISSN: 1538-7836
Language: English
Date published: 06/2003
Academic Unit: Stead Family Department of Pediatrics; Hematology/Oncology
Record Identifier: 9984353936802771

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