Abstract Number: PB0440
Meeting: ISTH 2021 Congress
Theme: Hemophilia and Rare Bleeding Disorders » Acquired Hemorrhagic Coagulation Disorders
Background: Acquired factor (F) V deficiency (AFVD) is a rare and potentially fatal coagulopathy with limited reported cases. Treatment aims to control bleeding and autoantibody eradication. Control of bleeding is challenging as commonly used hemostatic agents are not useful due to the low FV content. Herein we present a case of AFVD which combined use of recombinant activated factor VII (rFVIIa, Novoseven®; off-label use) and platelet transfusion (PTT) were effective to control a life-threatening hemorrhage.
Aims: Description of the use of combined therapy with PTT (as FV-source) and rFVIIa to support hemostatic effect to control bleeding in a severe case of AFVD.
Methods: Data were obtained prospectively after diagnosis and consent of the patient.
Results: A 77-year-old male with cirrhosis and several comorbidities presented severe hematuria, hemoglobin (Hb)= 10.9 g/dL, significant prothrombin time and activated partial thromboplastin time (aPTT) prolongation, normal fibrinogen plasma levels and negative lupus anticoagulant (Table 1). FV levels in the mixed test was 0% with a 2.4 BU/mL inhibitor titer. rFVIIa 70 µg/kg/3 h and methylprednisolone at a dose of 1.5 mg/kg/day were started. After 6 days of treatment and persistence of bleeding, PTT (1 pool/12 h) was administrated (5 pools in total). Hematuria disappeared after 48 h of PTT, stabilizing Hb thus PTT was suspended and gradual decrease of rFVIIa was also initiated until suspension 2 days later. At discharge, INR remained increased, with normal aPTT ratio and fibrinogen but 0.87 BU/mL inhibitor titer. After 18 days of corticosteroid therapy, inhibitor disappeared, and tapering was initiated. Patients is currently asymptomatic on prednisone tapering and oral vitamin K/24 h. INR (1.44) remains prolonged and FV= 53%
| Parameter | At admission | Day +1 | Day +2 | Day +4 | Day +9 | Day +12 | Day +18 | Day +25 |
| Hb (g/dL) |
16.1 | 13.4 | 11.5 | 10.9 | 10.8 | 11.6 | 13.8 | 14.8 |
| Platelets (x103/μL) |
168 | 154 | 159 | 206 | 178 | 111 | 82 | 81 |
| aPTT (s) | 214 | 195 | 111 | 86 | 49 | 42 | 31 | 27 |
| PT (s) | 68 | 58 | 39 | 38 | 25 | 27 | 21 | 18 |
| Fibrinogen (mg/dL) | 432 | 459 | 401 | 312 | 405 | 443 | 297 | 208 |
| FV (%) | UNK | UNK | 2 | 3 | 10 | 11 | 24 | 53 |
| Inhibitor titer (BU/mL) | UNK | UNK | 2.4 | 3.6 | 0.5 | 0.9 | 0.0 | UNK |
Laboratory parameters; PT: prothrombin time, aPTT: activated partial thromboplastin time, FV: factor V, UNK: unknown value.
Conclusions: PTT combined with rFVIIa hemostatic support were effective to control severe bleeding in a case of AFVD. Early use of PPT as FV source might improve outcome in this rare acquired condition.
To cite this abstract in AMA style:
Robles V, Dávalos CA, Serrano LM, Torres J, Fernández R, Chamorro P, Chávez MP, Fernández C. Life-threatening Bleeding in Acquired Factor V Inhibitor Can Be Controlled Combining Platelet Transfusion and Recombinant Activated Factor VII Administration [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/life-threatening-bleeding-in-acquired-factor-v-inhibitor-can-be-controlled-combining-platelet-transfusion-and-recombinant-activated-factor-vii-administration/. Accessed September 29, 2023.« Back to ISTH 2021 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/life-threatening-bleeding-in-acquired-factor-v-inhibitor-can-be-controlled-combining-platelet-transfusion-and-recombinant-activated-factor-vii-administration/