Basics to Brilliance: Haematology Podcast

Acquired Haemophilia A

Basics To Brilliance Season 2 Episode 11

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0:00 | 42:14

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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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