Acquired factor V inhibitor: a nation‐wide study of 38 patients. (20th January 2021)

• Record Type: Journal Article
• Authors: Goulenok, Tiphaine; Vasco, Claire; Faille, Dorothée; Ajzenberg, Nadine; De Raucourt, Emmanuelle; Dupont, Annabelle; Frere, Corinne; James, Chloé; Rabut, Elodie; Rugeri, Lucia; Schleinitz, Nicolas; Sacré, Karim; Papo, Thomas
• Other Names: Barbay Virginie investigator.; Becar Pierre investigator.; Laure Bigel Marie investigator.; Boehlen Françoise investigator.; Darnige Luc investigator.; de Raucourt Emmanuelle investigator.; Dubois Philippe investigator.; Dumas de la Roque Gerard investigator.; Flaujac Claire investigator.; Harlé Jean‐Robert investigator.; James Chloé investigator.; Jourde Noémie investigator.; Khelef Sabrina investigator.; Lacombe Yves investigator.; Langlois Michel investigator.; Lebreton Aurélien investigator.; Leflem Léna investigator.; Mamou Patrick investigator.; Massignon Denis investigator.; Meley Roland investigator.; Michel Marc investigator.; Mutuon Pierre investigator.; Pineau Vincent Fabienne investigator.; Reigner Antoine investigator.; Repesse Yohann investigator.; Rossignol Benoit investigator.; Ryman Anne investigator.; Tieulie Nathalie investigator.; Vasse Marc investigator.; Voisin Sophie investigator.
• Is Part Of: British journal of haematology. Volume 192:Number 5(2021)
• Page Start: 892
• Page End: 899
• Publication Date: 2021-01-20
• Subjects: acquired factor V inhibitor -- prothrombin time -- factor V inhibitor clearance -- bleeding; Hematology -- Periodicals; Blood -- Diseases -- Periodicals; 616.15
• DOI: 10.1111/bjh.17308
• Languages: English
• ISSNs: 0007-1048

Abstract

Summary: Acquired factor V inhibitor (AFVI) is an extremely rare disorder that may cause severe bleeding. To identify factors associated with bleeding risk in AFVI patients, a national, multicentre, retrospective study was made including all AFVI patients followed in 21 centres in France between 1988 and 2015. All patients had an isolated factor V (FV) deficiency <50% associated with inhibitor activity. Patients with constitutional FV deficiency and other causes of acquired coagulation FV deficiencies were excluded. The primary outcome was incident bleeding and factors associated with the primary outcome were identified. Thirty‐eight (74 [36–100] years, 42·1% females) patients with AFVI were analysed. Bleeding was reported in 18 (47·4%) patients at diagnosis and in three (7·9%) during follow‐up (7 [0·2–48.7] months). At diagnosis, FV was <10% in 31 (81·6%) patients. Bleeding at diagnosis was associated with a prolonged prothrombin time that strongly correlated with the AFVI level measured in plasma { r = 0·63, 95% confidence interval (CI) [0·36–0·80], P < 0·05}. Bleeding onset during follow‐up was associated with a slow AFVI clearance ( P < 0·001). The corresponding receiver operating characteristics curve showed that AFVI clearance was predictive of bleeding onset with an AFVI clearance of seven months with a sensitivity of 100% (95% CI: 29–100) and a specificity of 86% (95% CI: 57–98, P = 0·02). Kaplan–Meier analysis showed that AFVI clearance >7 months increased the risk of bleeding by 8 (95% CI: [0·67–97], P = 0·075). Prothrombin time at diagnosis and time for clearance of FV inhibitor during follow‐up are both associated with bleeding in patients with AFVI.