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Hamostaseologie 2011; 31(S 01): S20-S23
DOI: 10.1055/s-0037-1619744
Original article
Schattauer GmbH

Development of inhibitors in haemophilia

Ongoing epidemiological studyHemmkörperentwicklung bei HämophilenEine epidemiologische Studie
M. Serban
1   University of Medicine and Pharmacy “Victor Babes”, Timisoara
,
D. Mihailov
1   University of Medicine and Pharmacy “Victor Babes”, Timisoara
,
L. Pop
1   University of Medicine and Pharmacy “Victor Babes”, Timisoara
,
H. Ionita
1   University of Medicine and Pharmacy “Victor Babes”, Timisoara
,
E. Ursu
1   University of Medicine and Pharmacy “Victor Babes”, Timisoara
,
S. Talpos-Niculescu
1   University of Medicine and Pharmacy “Victor Babes”, Timisoara
,
L. Ritli
2   Paediatric Clinic Oradea
,
D. Baghiu
3   Hematology Clinic Targu-Mures
,
V. Uscatescu
4   Hematology Clinic Fundeni, Bucharest
,
C. Petrovanu
5   Haematology Clinic Iasi
,
P. Stancu
6   Clinical Hospital Craiova
,
D. Savescu
1   University of Medicine and Pharmacy “Victor Babes”, Timisoara
,
A. Cucuianu
7   Hematology Clinic Cluj Napoca
,
W. Schramm
8   Ludwig Maximillians University Munich
› Author AffiliationsThis work was supported by an unrestricted grant of Novo-Nordisk Haemophilia Foundation and we are indebted for this generous help.
Further Information

Publication History

received: 28 February 2011

accepted: 13 May 2011

Publication Date:
28 December 2017 (online)

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Summary

A number of 513 consecutive patients (494-haemophilia A and 19-haemophilia B) from eight haemophilia treatment centers have been investigated with Bethesda assay for the presence of factor VIII or IX inhibitors. The overall prevalence of inhibitors was 15.20%, 18.48% in severe, 5.60% in moderate and 12.24% in mild forms. The prevalence was higher than reported in most of the western countries. The age at start of substitution (p = 0.9775), the frequent switching of factor concentrates (p = 0.8931) were not relevant factors for the development of inhibitors. It is worth to be mentioned the unexpectedly occurrence of inhibitors in prior inhibitor negative (6/72) patients (during surgical interventions) probably due to their previous scarce substitution, occurrence which seems not being connected with the continuous infusion modality of factor VIII administration (p = 0.8341). In controversial situations, in the field of low titer (≤1 BU/ml) inhibitors for a reliable interpretation of the results the performance of recovery index and half-life time assessment of FVIII/IX was undertaken.

Zusammenfassung

In acht Hämophilie-Zentren wurden insgesamt 513 konsekutive Patienten (494 mit Hämophilie A und 19 mit Hämophilie B) für Hemmköper gegen FVIII/FIX mit der Bethesda-Methode untersucht. Die allgemeine Prävalenz von Inhibitoren lag bei 15,2%: 18,48% in schwerer, 5,6% in mittelschwerer und 12,24% in leichter Hämophilie. Die Bildung von Antikörpern tritt in höherer Zahl als in den meisten west europäischen Ländern auf. Beginn der Substitution (p = 0,9778), Frequenz von Änderungen von einem Faktorkonzentrat zu anderem (p = 0,8931) waren für die Entwicklung von Inhibitoren nicht relevant. Es ist zu vermerken die unerwartete Erscheinung von Inhibitoren bei vorher negativen Patienten (6/72) in Falle von chirurgischen Eingriffen, möglicherweise durch arme vorherige Substitution und nicht durch kontinuierliche Infusion von Faktor VIII (p = 0,8341) bedingt sein kann. In kontroversen Situationen, im Bereich von niedrigen Titern (≤1 BU/ml) wurde für eine korrekte Interpretation der Ergebnisse Recovery-Index und Halbwertszeit der Faktoren VIII und IX bestimmt.

Keywords

Haemophilia - inhibitors

Schlüsselwörter

Hämophilie - Inhibitoren
 

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