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Hemostatic management in an infant with neuroblastoma and severe hemophilia B with extended half-life recombinant factor IX fusion protein

Item Type:Article
Title:Hemostatic management in an infant with neuroblastoma and severe hemophilia B with extended half-life recombinant factor IX fusion protein
Creators Name:Cuntz, F. and Deubzer, H.E. and Schulte, J.H. and Nimtz-Talaska, A. and Eggert, A. and Holzhauer, S.
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.
Keywords:Coagulopathy, Disseminated Intravascular Coagulation, Hemophilia, Neuroblastoma, Pediatric
Source:Journal of Pediatric Hematology Oncology
ISSN:1077-4114
Publisher:Lippincott Williams & Wilkins
Date:3 March 2021
Official Publication:https://doi.org/10.1097/MPH.0000000000002109
PubMed:View item in PubMed

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