Inhibitors in Patients with Congenital Bleeding Disorders Other Than Hemophilia

Massimo Franchini; Giuseppe Marano; Carlo Mengoli; Vanessa Piccinini; Simonetta Pupella; Stefania Vaglio; Giancarlo Maria Liumbruno

doi:10.1055/s-0037-1607441

Seminars in Thrombosis and Hemostasis, Table of Contents

Semin Thromb Hemost 2018; 44(06): 595-603
DOI: 10.1055/s-0037-1607441

Review Article

Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Inhibitors in Patients with Congenital Bleeding Disorders Other Than Hemophilia

Massimo Franchini

¹Italian National Blood Centre, National Institute of Health, Rome, Italy

²Department of Hematology and Transfusion Medicine, Carlo Poma Hospital, Mantua, Italy

,

Giuseppe Marano

¹Italian National Blood Centre, National Institute of Health, Rome, Italy

,

Carlo Mengoli

¹Italian National Blood Centre, National Institute of Health, Rome, Italy

,

Vanessa Piccinini

¹Italian National Blood Centre, National Institute of Health, Rome, Italy

,

Simonetta Pupella

¹Italian National Blood Centre, National Institute of Health, Rome, Italy

,

Stefania Vaglio

¹Italian National Blood Centre, National Institute of Health, Rome, Italy

³Department of Clinical and Molecular Medicine, Sapienza University of Rome, Rome, Italy

,

Giancarlo Maria Liumbruno

¹Italian National Blood Centre, National Institute of Health, Rome, Italy

› Author Affiliations

Abstract

The most worrying complication of replacement therapy for severe hemophilia A and B is currently the occurrence of inhibitory alloantibodies against infused factor VIII and factor IX, respectively. Inhibitors compromise the management of hemorrhage in affected patients, with a considerable increase in complications, disability, and costs. While these alloantibodies have been extensively studied in the past years in hemophilia A and B, those occurring in patients with other inherited bleeding disorders are less well characterized and still poorly understood, mostly due to the rarity of these hemorrhagic conditions. This narrative review will deal with inhibitors arising in patients with inherited bleeding disorders other than “classical” hemophilia, focusing in particular on those developing in patients with congenital deficiency of coagulation factor V, factor VII, factor XI, and factor XIII.

Keywords

inherited bleeding disorders - alloantibodies - congenital - inhibitors

Full Text

References

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