Abstract
Abstract 717
Between 2003 and 2009 42 pregnant females were diagnosed with acquired haemophilia A (AHA) and included in the European Acquired Haemophilia A (EACH2) Registry. They constituted 8 % of the total number of admitted patients to the registry (n=501). In one female the diagnosis was made 36 days before delivery and the rest were diagnosed postpartum. One female had an autoimmune disorder, the others were without any associated disease. In 31 cases AHA occurred associated with the 1st pregnancy, in 7 the 2nd, in 2 the 3rd and in 2 the 4th pregnancy.
Data are given as median and inter quartile ranges:
| Reference | ||
|---|---|---|
| Age at pregnancy of interest | 34 (30–37) | |
| Days after delivery until diagnosis | 89 (25–180) | |
| Days between first abnormal APTT to diagnosis | 1 (0–8) | |
| Factor VIII at diagnosis | 2.5 (1–8) | 50–200 IU/dL |
| Anti Factor VIII antibody titre | 7.8 (2.4–31.3) | <0.4 BU/mL |
| Hb | 9.4 (7.5–12.9) | 11.7–17.0 g/dL |
| Reference | ||
|---|---|---|
| Age at pregnancy of interest | 34 (30–37) | |
| Days after delivery until diagnosis | 89 (25–180) | |
| Days between first abnormal APTT to diagnosis | 1 (0–8) | |
| Factor VIII at diagnosis | 2.5 (1–8) | 50–200 IU/dL |
| Anti Factor VIII antibody titre | 7.8 (2.4–31.3) | <0.4 BU/mL |
| Hb | 9.4 (7.5–12.9) | 11.7–17.0 g/dL |
All the diagnoses were triggered by bleeds. Cause of bleed: spontaneous 24, trauma 1, surgery 4, peri postpartum 15. Site of bleed: deep (musculoskeletal, retroperitoneal) 14, haemarthroses 2, mucosa 18, skin 19, CNS none. In 11 females the bleeds started on day of diagnosis. In 22 females the bleeds started between 0 and 7 days before diagnosis and in 20 females the bleeds were reported to have started more than 7 days before the diagnosis suggesting a significant delay in diagnosis. In 25 the bleed was considered as severe (a drop of Hb >2 g/dL or received red cell transfusions) – in 7 of these no haemostatic treatment was given. 21/41 of first bleed required haemostatic treatment, 12 with rFVIIa (recombinant activated factor VII), 5 aPCC (activated prothrombin complex concentrate), 2 FVIII concentrate, 2 desmopressin. In addition, 6 received antifibrinolytics. Ten needed red cell transfusions. The bleeds were controlled in all cases and there was no fatality.
First line immunosuppressive (IS) treatment:
| Regimen | CR* n (%) | NR** n (%) | Treatment changed n (%) | Days from start of IS | Relapse n (%) | ||
|---|---|---|---|---|---|---|---|
| Inhib <0.4 | FVIII >70 | IS stopped | |||||
| Steroids only n=31 | 22 (71) | 8 (26) | 1 (3) | 47 (29–112) | 40 (25–160) | 105 (63–236) | 2/20 (10) |
| Steroids and Cytotoxics n=6 | 5 (83) | 1 | 0 | 9, 35, 175, 261 | 12, 60, 175, 261 | 57, 60, 265, 363 | 0/5 |
| Steroids and rituximab n=2 | 2 (100) | 0 | 0 | 11, 76 | 11, 138 | 43, 53 | 0/2 |
| Regimen | CR* n (%) | NR** n (%) | Treatment changed n (%) | Days from start of IS | Relapse n (%) | ||
|---|---|---|---|---|---|---|---|
| Inhib <0.4 | FVIII >70 | IS stopped | |||||
| Steroids only n=31 | 22 (71) | 8 (26) | 1 (3) | 47 (29–112) | 40 (25–160) | 105 (63–236) | 2/20 (10) |
| Steroids and Cytotoxics n=6 | 5 (83) | 1 | 0 | 9, 35, 175, 261 | 12, 60, 175, 261 | 57, 60, 265, 363 | 0/5 |
| Steroids and rituximab n=2 | 2 (100) | 0 | 0 | 11, 76 | 11, 138 | 43, 53 | 0/2 |
CR = complete remission i.e. FVIII >70 IU/dL and no antibodies
NR = no complete remission
Summing up, AHA is a rare but severe bleeding condition in pregnancy/postpartum and is often overlooked. The diagnosis should be considered when screening tests show an abnormal APTT which indicates an immediate specific coagulation analysis. Acute bleeds are effectively treated with rFVIIa or aPCC. Immunosuppression with steroids to eliminate the anti FVIII inhibitor activity should be instituted as soon as possible after confirmation of AHA and this treatment seems to give a better response compared to older patients with AHA.
Nemes:Novo Nordisk:Tengborn:Novo Nordisk:Collins:NovoNordisk: Consultancy, Honoraria, The EACH2 registry was funded by Novonordisk; Baxter Healthcare: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding, Speakers Bureau. Baudo:NovoNordisk: Consultancy, Honoraria, NovoNordisk fund the EACH2 registry, Speakers Bureau; Bayer Healthcare: Honoraria, Speakers Bureau. Huth-Kuehne:NovoNordisk: Consultancy, Membership on an entity's Board of Directors or advisory committees, NovoNordisk fund the EACH2 registry; Baxter Healthcare: Consultancy, Membership on an entity's Board of Directors or advisory committees. Knoebl:Novo Nordisk: Consultancy, Membership on an entity's Board of Directors or advisory committees, NovoNordisk fund the EACH2 registry, Research Funding; Baxter Healthcare: Consultancy, Membership on an entity's Board of Directors or advisory committees, Research Funding. Marco:Novo Nordisk:Levesque:NovoNordisk:
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