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Hemostatic Management in an Infant With Neuroblastoma and Severe Hemophilia B With Extended Half-life Recombinant Factor IX Fusion Protein.
- 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.)
- Subjects :
- Abdominal Neoplasms blood
Abdominal Neoplasms diagnostic imaging
Abdominal Neoplasms therapy
Autografts
Factor IX pharmacokinetics
Humans
Infant
Male
Recombinant Fusion Proteins pharmacokinetics
Factor IX administration & dosage
Hemophilia B blood
Hemophilia B diagnostic imaging
Hemophilia B therapy
Hemostatics administration & dosage
Neuroblastoma blood
Neuroblastoma diagnostic imaging
Neuroblastoma therapy
Recombinant Fusion Proteins administration & dosage
Stem Cell Transplantation
Subjects
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