Basics to Brilliance: Haematology Podcast
Acquired Haemophilia A
Descripción
Feedback [https://www.buzzsprout.com/2344561/fan_mail/new] 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: basicstobrilliancehaem@gmail.com Insta: BasicstoBrilliance X: @basics_2_brill Send us your feedback!
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