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Hemostatic Management in an Infant With Neuroblastoma and Severe Hemophilia B With Extended Half-life Recombinant Factor IX Fusion Protein.

Authors :
Cuntz F
Deubzer HE
Schulte JH
Nimtz-Talaska A
Eggert A
Holzhauer S
Source :
Journal of pediatric hematology/oncology [J Pediatr Hematol Oncol] 2022 Jan 01; Vol. 44 (1), pp. e246-e249.
Publication Year :
2022

Abstract

In the rare co-occurrence of childhood cancer and severe hemophilia, hemostatic management is of paramount therapeutic importance. We present the case of an 11-month-old boy with severe congenital hemophilia B, who was diagnosed with metastatic high-risk neuroblastoma. He consequently developed paraneoplastic coagulopathy with life-threatening tumor hemorrhage and intracranial hemorrhage, showing central nervous system relapse. Management consisted of factor IX replacement with extended half-life factor IX fusion protein, adjusted to bleeding risk. Additional interventions included factor XIII, fibrinogen, fresh frozen plasma, tranexamic acid, and platelet transfusions. The half-life of factor IX products was markedly reduced requiring close factor IX monitoring and adequate replacement. This intensified treatment allowed chemotherapy, autologous stem cell transplantation, and GD2 antibody immune therapy without bleeding or thrombosis.<br />Competing Interests: The authors declare no conflict of interest.<br /> (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Details

Language :
English
ISSN :
1536-3678
Volume :
44
Issue :
1
Database :
MEDLINE
Journal :
Journal of pediatric hematology/oncology
Publication Type :
Academic Journal
Accession number :
33661164
Full Text :
https://doi.org/10.1097/MPH.0000000000002109