Cyclophosphamide vs rituximab for eradicating inhibitors in acquired hemophilia A: A randomized trial in 108 patients

Lévesque, H.; Viallard, J.F.; Houivet, E.; Bonnotte, B.; Voisin, S.; Le Cam-Duchez, V.; Maillot, F.; Lambert, M.; Liozon, E.; Hervier, B.; Fain, O.; Guillet, B.; Schmidt, J.; Luca, L.E.; Ebbo, M.; Ferreira-Maldent, N.; Babuty, A.; Sailler, L.; Duffau, P.; Barbay, V.; Audia, S.; Benichou, J.; Graveleau, J.; Benhamou, Y.

doi:10.1016/j.thromres.2024.03.012

Cited by 3 publications

(1 citation statement)

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“…Over the last 20 years, the anti-CD20 monoclonal antibody rituximab has been effective in the management of certain autoimmune diseases and cancers. There have been retrospective studies performed on treating AHA patients who have high Bethesda unit (>100BU/ml) with rituximab, and in these patients, rituximab therapy has been an effective agent in achieving a similar response (complete response or partial remission) with a better safety profile than other immunosuppressive agents [ 19 - 21 ]. Intravenous immunoglobulin (IVIG) therapy and single-agent mycophenolate mofetil or cyclophosphamide were also investigated, but these medications need comparative study with currently recommended regimens [ 22 , 23 ].…”

Section: Discussionmentioning

confidence: 99%

A Rare Case of Hemophilia: Acquired Factor VIII Deficiency

Winn,

Mohsin,

Peeke

2024

Cureus

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Unlike hereditary hemophilia, acquired hemophilia is a rare life-threatening bleeding disorder that occurs in a person who has no personal or family history of bleeding episodes. It usually presents with new-onset subcutaneous/joint/muscle bleeding causing ecchymoses and hematomas, hematuria, GI bleeding, retroperitoneal bleeding, or rarely acute intracranial hemorrhage in elderly individuals. The diagnosis involves assessing prolonged activated partial thromboplastin time (aPTT) and conducting mixing studies after ruling out lupus anticoagulants and interfering substances such as heparins. Management consists of controlling hemostasis using recombinant activated factor VII (rFVIIa) and activated prothrombin complex concentrates (aPCC), along with eradicating autoantibodies against factor VIII from the body system using immunosuppressive therapies. Due to the risk of uncontrolled bleeding in individuals who were previously normal, delayed diagnoses and recurrences are not uncommon, potentially resulting in unfavorable outcomes.

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