Successful Treatment of Acquired Factor v Inhibitor With Severe Hemorrhagic Manifestations by Steroid Pulse Therapy and Platelet Transfusions

Tomisaka, Ryuya; Hanaoka, Nobuyoshi; Mine, Azusa; Nakajima, Shiho; Horibata, Yoko; Matsunami, Misako; Nakamura, Yoshinobu; Oishi, Hiroaki; Sawada, Takashi

doi:10.3925/jjtc.61.539

Cited by 2 publications

(2 citation statements)

References 13 publications

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“…This is likely because FFP contains only 1 U/mL of FV and is easily overwhelmed by FV neutralizing autoantibodies. The repeat administration of FFP may be risky in patients with ongoing bleeding because of possible circulatory overload, 54 aggravation of bleeding symptoms, 55 and coagulation abnormalities such as an increase in FV inhibitor titer.…”

Section: Hemostatic Treatmentmentioning

confidence: 99%

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A Review of Coagulation Abnormalities of Autoimmune Acquired Factor V Deficiency with a Focus on Japan

Ichinose

Osaki

Souri

2021

Semin Thromb Hemost

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Coagulation factor V (or FV for the purpose of medical safety) is an essential cofactor of coagulation factor X in the common pathway of coagulation; severe FV deficiency leads to a bleeding tendency. Although both congenital and acquired FV deficiencies are widely recognized, FV deficiency also presents as an autoimmune disorder. A nationwide survey on autoimmune coagulation factor deficiencies (AiCFDs) conducted in Japan by our Japanese Collaborative Research Group identified 24 new patients with autoimmune FV deficiency (AiFVD) in the past 5 years. Furthermore, our extensive literature search confirmed that 177 AiFVD cases have been reported in previous articles published from Japan. Patients with AiFVD in Japan were predominantly men, with age similar to those with other AiCFDs. AiFVD was confirmed as a relatively mild type of bleeding diathesis, associated with lower mortality rate than that for AiFVD and other AiCFDs reported in previous studies. Patients with AiFVD had variable FV inhibitor titers and both neutralizing anti-FV autoantibodies and nonneutralizing counterparts. Although spontaneous resolution occurs in some patients, timely initiation of hemostatic and immunosuppressive therapies helps arrest the bleeding and eliminate anti-FV antibodies, resulting in a high cumulative recovery rate. Immunological anti-FV antibody detection is recommended to avoid missing AiFVD cases for the presence of nonneutralizing anti-FV autoantibodies. Further investigation is necessary to clarify the long-term prognosis and optimal management of AiFVD.

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Section: Hemostatic Treatmentmentioning

confidence: 99%

“…Similar overall and spontaneous resolution rates were previously reported in 69.2% (54/78) and 15.4% (12/54) of patients with FV inhibitor, respectively. 9 A total of 10 (5%) patients showed disease deterioration after reinitiating the antibiotic therapy, 29 administering FFP infusion, 55 or tapering the dose of prednisolone. 49 54 58 59…”

Section: Prognosismentioning

confidence: 99%