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Emicizumab in two patients with acquired haemophilia A – case report Cover

Emicizumab in two patients with acquired haemophilia A – case report

The Journal of Haemophilia Practice
Volume 11 (2024): Issue 1 (January 2024)
By: Milly Zhao,  Thomas Kartika,  Corey Witenko,  Jessica Snead,  Maria T DeSancho and  Alana Ciolek  
Open Access
|Aug 2024

Figures & Tables

The cases of two patients with acquired haemophilia A treated with emicizumab suggest its early initiation as part of frontline therapy may reduce episodes of bleeding and reduce the need for bypassing agents
The cases of two patients with acquired haemophilia A treated with emicizumab suggest its early initiation as part of frontline therapy may reduce episodes of bleeding and reduce the need for bypassing agents

Figure 1.

Timeline of events in care of the first patient Days are with respect to the initial presentation. The patient’s coagulation parameters and FVIII levels and inhibitor are shown in relation to administration of haemostatic agents and immunosuppressive therapy (IST) including rituximab and prednisone. On day 67 and 74, the Factor VIII levels on the one-stage assay (FVIII:h) were 273 and 278%, respectively [not shown]. Patient had debridement done on day 70 and initiated on recombinant factor VIIa (rFVIIa), subsequently developing a deep vein thrombus on day 76. aPTT = activated partial thromboplastin time; FVIII:b = chromogenic FVIII measurement using bovine reagent; aPCC = activated prothrombin complex concentrate; LMWH = low molecular weight heparin
Timeline of events in care of the first patient Days are with respect to the initial presentation. The patient’s coagulation parameters and FVIII levels and inhibitor are shown in relation to administration of haemostatic agents and immunosuppressive therapy (IST) including rituximab and prednisone. On day 67 and 74, the Factor VIII levels on the one-stage assay (FVIII:h) were 273 and 278%, respectively [not shown]. Patient had debridement done on day 70 and initiated on recombinant factor VIIa (rFVIIa), subsequently developing a deep vein thrombus on day 76. aPTT = activated partial thromboplastin time; FVIII:b = chromogenic FVIII measurement using bovine reagent; aPCC = activated prothrombin complex concentrate; LMWH = low molecular weight heparin

Figure 2.

Timeline of events in care of the second patient Days are with respect to the initial presentation. The patient’s coagulation parameters and Factor VIII (FVIII) levels and inhibitor are shown in relation to administration of haemostatic agents and immunosuppressive therapy (IST) including intravenous immunoglobulin, prednisone, rituximab, and cyclophosphamide. During recurrence, the patient received 3 doses of emicizumab. Inhibitor level became undetectable three months post relapse. aPTT = activated partial thromboplastin time; FVIII:h = FVIII measurement via one-stage assay; FVIII:b = chromogenic FVIII measurement using bovine reagent; aPCC = activated prothrombin complex concentrate
Timeline of events in care of the second patient Days are with respect to the initial presentation. The patient’s coagulation parameters and Factor VIII (FVIII) levels and inhibitor are shown in relation to administration of haemostatic agents and immunosuppressive therapy (IST) including intravenous immunoglobulin, prednisone, rituximab, and cyclophosphamide. During recurrence, the patient received 3 doses of emicizumab. Inhibitor level became undetectable three months post relapse. aPTT = activated partial thromboplastin time; FVIII:h = FVIII measurement via one-stage assay; FVIII:b = chromogenic FVIII measurement using bovine reagent; aPCC = activated prothrombin complex concentrate
DOI: https://doi.org/10.2478/jhp-2024-0016 | Journal eISSN: 2055-3390
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Language: English
Page range: 92 - 98
Published on: Aug 2, 2024
Published by: Haemnet Ltd
In partnership with: Paradigm Publishing Services
Publication frequency: 1 issue per year
Keywords:
Acquired haemophilia,
Emicizumab,
Intracranial haemorrhage,
Inhibitors,
Recombinant factor VIIa,
Case report
Related subjects:
Medicine,
Basic medical science,
Basic medical science, other,
Clinical medicine,
Clinical medicine, other,
Pharmacy,
Pharmacology

© 2024 Milly Zhao, Thomas Kartika, Corey Witenko, Jessica Snead, Maria T DeSancho, Alana Ciolek, published by Haemnet Ltd
This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

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