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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
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