Table 2.
Baseline characteristics reported across included studies
| Primary publication | Study design | No. of patients treated | Mean age | Sex (% male) | Mean FVIII level (IU/dL) | Mean inhibitor titre (BU/mL) | Cause of bleeding (%) | Bleeding site (%) | Severity of bleeding (%) | Underlying conditions (%) | Ancillary therapies (%) | Patients with AH treated (%) | ||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Traumatic | Spontaneous | Skin | Muscle | Mucosa | Head | Joint | GI tract | Multiple sites | Other | Severe | Non-severe | Autoimmune | Malignancy/cancer | Post-partum | Idiopathic | Anti-fibrinolytic agents | Red blood cells | rFVIIa alone | rFVIIa + other haemostatic agents | |||||||
| Hay 1997 [30] | EAP | 38 | 59a | 50 | – | 43a | – | – | – | 51.3b | – | – | 9.0b | 20.5b | – | 19.2b | – | – | – | – | – | – | 66b | – | – | – |
| Baudo 2004 [26] | ROB | 15 | 72a | 60 | – | 15a | 4.3b | 73.9b | 35b | 55b | – | – | 5b | – | – | 5b | 95b | – | 7 | 13 | – | 80 | 6.7 | 66.7 | ||
| Dehmel 2008 [25] | POB | 10 | 76 | 40 | 2a | 10a | – | – | 10 | – | – | – | – | 60 | 30 | – | – | – | – | – | – | 20 | 40 | – | – | |
| Luis 2010 [23] | ROB | 11 | – | – | – | – | – | – | – | – | – | – | – | – | – | – | – | – | – | – | – | – | – | – | 100 | |
| Dusseldorf study (Scharf 2011a [31]) | POB | 35 | – | 68.5 | – | – | – | – | 62 | 10.3 | – | – | – | 13.8 | – | 13.8 | 45.7 | – | 23 | 8.6 | 2.9 | 40 | – | – | 91.4 | 8.6 |
| EACH2 registry (Baudo 2012 [7]) | Registry | 174 | 73a | 54.1 | 2a | 15.0a | 24.1 | 75.9 | 15.2 | 59.1 | 18.8 | 3.1 | 3.1 | – | 0.7 | – | 89.8 | 10.1 | 14.1c | 11.5c | 8.9c | 54.4c | 17 | 54.4c | – | – |
| HTRS Registry (Ma 2012 [18])c | Registry | 68 | 74 | 49 | – | 56d | 21.6b | 68.4b | 38.1b | 20.9b | 32.3b | 2.9b | 15.1b | – | – | 10.1b | – | – | 28.3 | 14.4 | 3.4 | – | – | 20.9b | 64b | 36b |
| SACHA registry (Borg 2013 [2])c | Registry | 28 | 76.7a | 61 | – | 16a | – | – | 61 | 34 | 6 | 1 | 7 | 5 | – | 6 | 39.3 | – | 14.63 | 19.5 | 7.32 | 55 | – | 35.4 | 28 (93) | 2 (7) |
| Japanese PMS (Seita 2013 [21])e | POB | 132 | 67.9f | 57f | 4.1f | 101.1f | – | – | 12b | 40b | – | 4b | 10b | 3b | – | 35b | 47 | – | 26.5f | 12f | 0f | – | – | – | 100 | – |
| AHS (Lentz 2014 [28])c | Registry | 65 | 66.6 | 41 | – | 154.5 | – | 85 | – | – | – | – | – | – | – | – | – | – | 34 | 12 | 5 | – | – | – | – | – |
| Zhang 2015 [29]e | ROB | 32 | 57.3 | 33.3 | 5.5 | 11.3 | 50 | 50 | 25 | 33.4 | 25 | 8.3 | – | – | 8.3 | – | 50 | 50 | 19.6c | 12.5c | 1.8c | – | 33.3 | 8.3 | 12 (37.5)c | 20 (62.5)c |
| GTH registry (data on file) [24] | Registry | 61 | 71.43 | 62 | 3.19 | 14.6a | – | – | 29.8b,c | 42.6b,c | – | 0.7b,c | 1.7b,c | 9.3b,c | – | 15.9b,c | 54.3b | 45.7b | 20c | 13c | 5c | 67c | – | – | – | – |
aMedian value; bBleed-level data; cStudies in which overall baseline data were captured, as baseline characteristics for patients receiving rFVIIa were not reported separately; dMedian highest inhibitor; eData provided for rFVIIa-only treated group; frFVIIa monotherapy/combination therapy. Underlying disease and bleed severity in Dusseldorf study is reported for subpopulation of 29 patients in Gheisari 2010 [35]. AH, acquired haemophilia; AHS, acquired haemophilia surveillance; EACH2, European Acquired Haemophilia; EAP, extended access program; FVIII, factor VIII; GI, gastrointestinal; Hb, haemoglobin; HTRS, Haemostasis and Thrombosis Research Society; POB, prospective observational study; rFVIIa, recombinant factor VIIa; ROB, retrospective observational study; SACHA, Surveillance des Auto antiCorps au cours de l’Hémophilie Acquise