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Acquired Haemophilia A
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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
'Basics to Brilliance: Haematology Podcast' has been accredited for CPD credit by the Royal College of Pathologists UK.
Medical professionals and clinical scientists holding career-grade positions, who are registered with any of the Royal Colleges for CPD, will be eligible to earn 1 credit for every hour of learning.
Email: [email protected]
Insta: BasicstoBrilliance
X: @basics_2_brill
Send us your feedback!
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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
'Basics to Brilliance: Haematology Podcast' has been accredited for CPD credit by the Royal College of Pathologists UK.
Medical professionals and clinical scientists holding career-grade positions, who are registered with any of the Royal Colleges for CPD, will be eligible to earn 1 credit for every hour of learning.
Email: [email protected]
Insta: BasicstoBrilliance
X: @basics_2_brill
Send us your feedback!