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00:52 Intro and chuckles
01:40 Case study: 75M, left calf swelling, put on DOAC, 24 hrs later haematoma and deep bleed on CT
06:00 General information
Elderly (>65), Mortality 8-40%
Common presentations: GI and UG bleeding, Retroperitoneal and muscle bleeds (compartment syndrome)
Ptegnancy, TTP, Malignancy (15%), Autoimmune disease (17%)
08:56 Pathogenesis and diagnosis: AutoAb against F8
*Bethesda units do not correlate with bleeding phenotype in Acquired HA- second orfer kinetics*
History
APTT, PT (isolated raised APTT)
Mixing studies: 50/50 or 80/20 mix
Factor Assays (**Intrinsic**)
Decreased Factor VIII + Non-paralellism -> Bethesday Assay
20:20 Non-clotting investigations
22:05 Treatment
MDT + Comprehensive Care Center escalation
RICE., TXA, Bypassing agents
Limit iatrogenic bleeding
Review medications
Pregnancy: birth plan!!! inhibitor can cross palcenta
Steroid +/- Cyclophosphamide
27:10 Bypassing Agents in Acquired Haemophilia A
FENOC + EACH2 study: FEIBA vs NovoSeven = No difference in bleeding/thrombosis rates- more info at 33:25 for EACH2
Obizor can be titrated according to response whereas FEIBA and NovoSeven cannot
Emicizimab +/- Immunosuppression = Not currently licesnsed in the UK
32:25 Inhibitor eradication
Mean time to remission: 5 weeks
Good prognostic markers: FVIII 1 or more, Inhibitor titre < 20
EACH 2 Study: Steroids -> Steroids + Cyclophosphamide -> Steroids + Cyclo + Rituximab
Biggest cause of death: infection
36:45 Follow up
Weekly FVIII levels and inhibitor monitoring till remission then monthly for 6 months then 2-3 monthly for a year
Planned procedure; FVIII level
38:45 Golden Nuggets
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