High rate of spontaneous inhibitor clearance during the long term observation study of a single cohort of 524 haemophilia A patients not undergoing immunotoleranceReport as inadecuate




High rate of spontaneous inhibitor clearance during the long term observation study of a single cohort of 524 haemophilia A patients not undergoing immunotolerance - Download this document for free, or read online. Document in PDF available to download.

Journal of Hematology and Oncology

, 6:63

First Online: 30 August 2013Received: 15 July 2013Accepted: 27 August 2013DOI: 10.1186-1756-8722-6-63

Cite this article as: Tagariello, G., Iorio, A., Matino, D. et al. J Hematol Oncol 2013 6: 63. doi:10.1186-1756-8722-6-63

Abstract

BackgroundThe natural history of inhibitors in patients with haemophilia A not undergoing immune tolerance induction ITI is largely unknown. A recent randomized controlled trial suggests that the higher the FVIII dose used for ITI, the faster the clearance and the lower the rate of bleeding, without any difference in the rate of tolerance. We aimed at assessing the rate of spontaneous inhibitor clearance in a large cohort of patients not undergoing ITI.

MethodsA retrospective analysis of anti-FVIII inhibitors of long-term registry data in a single centre cohort of 524 haemophilia A patients considered for synovectomy was performed. Patients were tested for inhibitors before and 15 days after any and each surgical episode and thereafter did not undergo immune tolerance at any time.

ResultsThe cumulative incidence of inhibitors overall was 34% 180 out of 524 with the highest percentage of 39% 168 out of 434 in severe patients which represented 83% of the cohort. Among the 180 inhibitor patients: 63 had permanent inhibitors; 70 fulfilled current criteria for transient inhibitors but a third category of 47 additional patients cleared the alloantibody spontaneously in >6 months. At logistic regression, both the inhibitor titre and the gene mutation were shown to predict time to clearance.

ConclusionsSpontaneous clearance of inhibitors over variable time in the absence of ITI treatment was found in up to 2-3 of the cases.

KeywordsHemophilia A Inhibitors ITI Electronic supplementary materialThe online version of this article doi:10.1186-1756-8722-6-63 contains supplementary material, which is available to authorized users.

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Author: Giuseppe Tagariello - Alfonso Iorio - Davide Matino - Donata Belvini - Roberta Salviato - Roberto Sartori - Paolo Radossi

Source: https://link.springer.com/







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