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Emicizumab in two patients with acquired haemophilia A – case report

,

Kartika, Thomas

,

,

,

Maria T DeSancho

DeSancho, Maria T

et

02 août 2024

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

The Journal of Haemophilia Practice

Édition 11 (2024): Edition 1 (Janvier 2024)

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Catégorie d'article: Case Study

Publié en ligne: 02 août 2024

Pages: 92 - 98

DOI: https://doi.org/10.2478/jhp-2024-0016

Mots clés
Acquired haemophilia, Emicizumab, Intracranial haemorrhage, Inhibitors, Recombinant factor VIIa, Case report

© 2024 Milly Zhao et al., published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

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

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

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

Langue:: Anglais

Périodicité:: 1 fois par an
Sujets de la revue:: Médecine, Sciences médicales de base, Sciences médicales de base, autres, Médecine clinique, Médecine clinique, autres, Pharmacie, Pharmacologie

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