Basics to Brilliance: Haematology Podcast

Basics To Brilliance
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Welcome to Basics to Brilliance, the podcast created to supplement & bolster your knowledge of Haematology.Featuring a two way, non-didactic conversational-style Q&A between the SpR and SHO, this podcast will be your pocket companion no matter where you are.We aim to cover: - Malignant and non-malignant topics - Science/lab detail - UK guidelines, hallmark trials and how these translate into clinical practice - Future research directions - The whole syllabus for FRCPath part 1 All readily accessible and completely free of charge!For every budding haematologist out there, we hope this podcast aids you in your endeavours and fills you with interest and excitement for the brilliant world of Haematology. Warmest Regards,Dr. EverdenDr. FaseyDr. Jafri Disclaimer: This podcast is intended as a revision aid and should not be used for the medical management of patients. Guidelines in the initial episodes span 2023/2024. We aim to update our content in accordance with the most recent available guidelines when possible.  This podcast is CPD accredited by the Royal College of Pathologists UK 

  1. NOV 30

    Haemophilia A: Inhibitors

    Feedback 00:52 Intro 02:10 Definition Common: Alloantibody neutralizes FVIIIRare: causes increased clearance of FVIII1/3 of Severe Haemophilia A patientsMedian time 10-15 emergency dosesRF: Mutation types (INSIGHT study), 60, African/Hispanic, HIVneg, large rFVIII doses, FVIII + inflammatory stimulus07:05 Inhibitor classifications Titres: Low (5 BU)Responder: Low vs HighTime: Dependent (FVIII inh.) vs Independent (FIX inh.)09:50 Presentation in practice Treatment failure, change in bleeding pattern, anaphylaxisScreening: prior to invasive procedures, before/after treatment changesRoutine surveillance:Mild- moderate: Yearly + 2-3 wks after emergency treatmentSevere: Every 3rd emergency dose or 3 monthly13:55 Tests (needs repeat) Mixing studyFVIII assay (48 hours post dose)Bethesda assay (if 80-100% residual FVIII = no inhibitor)Specialist: ELISA, In-vivo recoveryMost sensitive: FVIII half-life studiesInhibitor Assays (Bovine chromogenic assay)19:45 Preventative measures? Mild to moderate: DDAVP when possibleSevere: prophylaxis 23:40 Treatments Bypass agent: skips intrinsic pathway, straight to extrinsic F.I.B.A: activated PCC (II, VIII, IX, X)Onset 15-30 minsDose: 50-100IU/kgHalf life 8-12 hrsNB: Plasma derived: FVIII contamination, infectionContraindicated: EmicizimabNovoSeven: activated rFVIIaPeak 15 minsDose: 270ug/kg, fixed dose, can’t titrateNB: Half life 2 hours, can’t titrateObizor: Porcine rVIIIPeak 30 minsDose: 200IU/kg and titrateablePorcine Bethesda before use34:35 Prophylaxis EmicizumabImmune tolerance induction pts: Emicizumab, NovoSeven > FIBABreakthrough bleeding: increase frequency38:22 Immune Tolerance Induction: start ASAP if inhibitor present UKHCDO: Long term FVIII tolerance induction and maintenance is key for severe Haemophilia A - don't rely on Emicizumab Success rate: 70%, consistent treatment, fewer emergency doses prior to starting, Historic peak titre 500 BU titres46:20 International Immune Tolerance Study (Hay, DiMichele)- Blood 2012 47:50 Monitoring and Response Assessment  Success: FVIII half-life  >7 hours BU negativeMeasurable trough levels at 48 hoursFailure: escalated to max rFVIII but still uptrending titres or fall of 20% in 6 months (alternative systemic agents)NB: rule out intercurrent infection50:33  VerITI-8 trial  51:28 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!

    54 min
  2. Haemophilia A: Management Basics

    NOV 16

    Haemophilia A: Management Basics

    Feedback 00:52 Intro 02:35 Structure of Haemophilia A care 05:10 Key aspects of management (On-demand vs prophylaxis) 07:00 Prophylaxis: reduce death rates from ICH and reducing joint bleeds - Primary prophylaxis: before the 2nd joint bleed Severe haemophiliaAny child spontaneous ICHModerate haemophilia A (1-3 IU/dL)- Secondary prophylaxis: After the 2nd joint bleedLimit joint damage and maximize long term functionESPRIT trial- Tertiary prophylaxis: If joint disease already establishedSlow progression, reduce pain and improve QOLSPINART study13:10 Phases of treatment in Primary Prophylaxis - Modify dose during according to needs at that stage of life - By adulthood, 30% of severe patients can safely stop primary prophylaxis! 15:50 Prophylactic medications - IV Recombinant FVIII, 1 IU/kg increases by 2 IU/dL (2%) - Half life: 8-12 hrs  - Primary Prophylaxis: (needs CVC) Aim trough level 1-3 IU/dL25-40 IU/kg approx 3-4x per weekTitrate clinically which is individual to the patient- Extended half life...ratio of regular:half-life should be at least 1:1.3 - Cannot START them on this as can cause inhibitor 25:40 Efanesoctocog (EFA) only needs once weekly IV dosing - Very extended half life - XTEND 1 and XTEND-KIDS trials 27:52 Emicisimab: Bi-specific Ab, SC, half life 30 days - Binds FIXa to FX thereby replacing FVIII - Phenotypically makes patients have mild haemophilia A - Used for ANY haemophilia with inhibitor OR severe haemophilia without an inhibitor - HAVEN 3 study - Breakthrough bleeds ?management challenges at home - FIBA can cause MAHA (don't use together)  - Thrombosis risk - Reduces APTT and interferes with measuring FVIII and inhibitor 45:50 On-demand therapy (inhibitor dependent) 47:50 Joint bleeds - Moderate bleed: Aim peak of 50- 60 IU/dL - Severe bleed: Aim peak of 60-80 IU/dL - Daily dosing - Assess within 15 mins , treat within 30 mins - TXA + analgesia, PT + PRICE  52:20 Other bleeds - Peak 80-100 IU/dL: Iliopsoas, ICH, GI bleeds, Neck/throat - Deep cut: aim peak 50 IU/dL - Keep at peak for 1-3 days then 50% decrease in peak level for the next week 54:10 Case-study: 24M, swollen knee, FVIII 0.3 IU/L - Patients usually have an emergency plan - Assume severe if no info. 01:00:15 Case study: 38M, Appendicectomy, FVIII 30 IU/dL - Hari tricks David, David is tricked - Give DDAVP because rFVIII can cause an inhibitor 01:03:05 DDAVP (Vasopressin)- IV/SC- 3-5 fold increase of FVIII - 0.3micrograms/kg - Releases a pool of FVIII from endothelium *lung*- tachyphylaxis - Check protocol, side effects and contraindications 1:08:36 Management planning in elective surgery (MDT) - TXA! - Calculate dose of Recomb. FVIII - Check levels at 15mins(pre-op), 4hrs (post-op), next morning - May need VTEp 01:12:05 Comprehensive Care Centre Annual Review checklist- exam pearl NB: Target joint- 3 or more bleeds into one joint in a 6 month '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!

    1h 25m
  3. Haemophilia A: Diagnosis & Investigations

    NOV 9

    Haemophilia A: Diagnosis & Investigations

    Feedback 00:52 Intro 02:00 What is Haemophilia A? 03:00 Factor VIII, Pathogenesis of Haemophilia A 07:10 Structure of Factor VIII (exam pearl) 300kDHeavy (A1 A2, B) + Light Chain (A3, C1, C2) bound by a metal ions *Calcium*A subunits are 30% homologousB subunit (variable region) is cleaved by thrombin to get Factor VIIIaC1 and C2 help bind to VWFGood to r/o VWF10:55 Epidemiology and history taking- X-linked recessive Factor VIII is (mostly) feminist.....Turners syndrome, Androgen Insensitivity syndrome, Consanguinity etc. can present with HA  Hari talks about cats 50% of severe haemophilia A have an Intron 22 mutation (non-coding)1/3 de-novo mutationsAmount of Factor VIII negatively correlates with severity (less VIII= more severe)21:55 Presentation of Haemophilia A Spontaneous Joint and Muscle bleeds + Op bleedingYounger presentation = more severeSpontaneous ICH in baby/child inc. in birth traumaThink about On/Off recurrent oromucosal bleeding (can become anaemic)30:00 Non-accidental injury in pre-mobile child- check BSH guidance** 33:35 Diagnosis Factor VIII 40 IU/dL in general (units useful to think as percentages- 40%)Mild 6-40%Moderate 1-5%Severe 1%, presents 2 yo and diagnosed 4 yo35:50 Investigations Bleeding and Family HxFBC, PT/APTT (expect normal PT, abnormal APTT correcting with mixing studies)severe  eg. APTT 100moderate eg. APTT 60-100Factor VIII, IX, XI , XII assay (intirnsic)1 stage and 2 stage chromogenic assay VWF assay (antigen and activity)VW Factor VIII binding assay to r/o VWF 2MGenetic testing (HCDO)- mutational analysis. Can inform likelihood of inhibitor formation and **female carrier identification**Factor V and VIII can be co-inherited in an autosomal inherited pattern*** Biggest cause of death is intracranial death *** *** Biggest cause of disability is target joint deformation *** 47:52 Summary  '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!

    49 min
  4. Factor XIII (13), Fibrinolysis & Thrombolysis

    JUL 12

    Factor XIII (13), Fibrinolysis & Thrombolysis

    Feedback 01:45 Case: Neonate with repeated umblical bleeding. IC haemorrhage.  Normal Factors (so far), Normal VW screen, Normal FBC and normal film. 05:35 Factor XIII (13): function and presentations in deficiency  09:45 Testing, testing!  ELISA/Ammonia Release Assay then a Mutational Analysis Honarable mention: Clot Solubility Assay16:00 Fibrinolysis definition and pathway 21:00 Activators of Fibrinolysis: tPA vs. uPA  23:23 Inhibitors of Fibrinolysis: PAI-1, PAI-2, TAFI, α2-antiplasmin, and Factor XIII (13) 26:28 Thrombolysis: defintion, examples and uses 32:20 Case: Thromboembolic stroke, thrombolysis 24 hours prior, haemorrhagic transformation 36:19: Tranexamic Acid (Hari’s favourite drug) Honarable mention: Dr Utako Okamoto and the WOMAN trialCRASH 2 and CRASH 3 trialsHALT-IT trial41:22 D-Dimer  44:00 Summary '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!

    46 min
  5. Inhibitors in Haemostasis

    JUN 7

    Inhibitors in Haemostasis

    Feedback 00:52 Intro: definition and prompts 04:25 Intro to Case 1: Haemophilia B, 3rd dose of BeneFix Anaphylaxis  06:03 Intro to Case 2: Severe Haemophilia A, joint bleed, non-responsive to emergency Factor VIII 07:30 Initial screening tests  NB: Inhibitors: Time Dependent vs. Immediate Acting14:54 Flash examples of Mixing Study importance 16:35 Bethesda Assay  **Bethesda Studios made the Elder Scrolls and Fallout games: they Inhibit Haider from doing any work** 28:45 Bethesda Assay Summary 30:00 Nijmegen Modification of Bethesda Assay 34:30 Heat Treatment of Bethesda Assay  36:25 ELISA Assay  40:30 Auto vs Allo Antibodies 42:00 Kinetics of a Time Dependent Inhibtor  44:45 Case 1 results 45:50 Case 2 results 51:00 Case 3 (real-life scenario): young girl, subdural bleed after fall, deranged clotting found incidentally  59:30 Summary  '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!

    1h 3m
  6. Heparin & the Anti-Xa Assay

    MAY 25

    Heparin & the Anti-Xa Assay

    Feedback 00:52 Intro (shoutuout to the BSH anticoagulant monitoring guidelines) 02:15 Practical relevance of testing and monitoring anticoagulants  07:30 Heparin: The Basics  09:00 Unfractionated Heparin vs. Low Molecular Weight Heparin  10:24 Mechanism of action of Heparin (UfH vs. LMWH) LMWH: more Anti-Xa activityUfH Anti-IIa acitivity = Anti-Xa activity15:30 Pharmacokinetic differences (UfH vs. LMWH) 23:28 Unfractionated Heparin uses and monitoring 34:34 Anti Xa Assay 42:32 Activated Clotting Time (POCT) 46:56 Anti Xa in LMWH: Practical considerations 52:03 LMWH uses and dosing 55:30 Summary '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!

    59 min
  7. Thrombin Time & Fibrinogen Assays

    MAY 18

    Thrombin Time & Fibrinogen Assays

    Feedback 00:54 Intro and table of contents 1:48 Case 1: pre-op, prolonged PT and ++ prolonged APTT…thrombin time done 03:44 Case 2: post-op, normal PT and prolonged APTT…thrombin time done 04:46 Thrombin Time definition and ingredients (its all about the fibrinogen!) 10:45  Differentiating causes of prolonged thrombin time- protamine, reptilase, ecarin 17:24 Case 3: Major Haemorrhage (Variceal), due OGD, Derived PT Fibrinogen normal 20:45 Clauss Fibrinogen- methodology, causes of change and treatment of deranged fibrinogen 30:09 Derived PT Fibringoen 31:20 ELISA, TEG and ROTEM (brief) 33:07 Summary  36:56 David’s request (email us your questions!) '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!

    38 min
  8. Mixing Studies & Factor Assays

    APR 27

    Mixing Studies & Factor Assays

    Feedback 0:52 Intro and table of contents 2:36 CASE 1- Infection, due surgery and a prolonged APTT  ft. a refresher on APTT prolongation 08:00 Mixing studies- definition and uses ft. Hari’s exam nugget 17:00 Factor assays (1 stage, 2 stage and chromogenic assays) ft. David’s humorous humility 43:30 David applies his new-found knowledge to our first case 45:45 CASE 2-  Infection, hx of weight loss and bleeding and a prolonged APTT  47:36 Summary    https://practical-haemostasis.com/Factor%20Assays/1_stage_pt_factor_assay.html https://practical-haemostasis.com/Factor%20Assays/chromogenic_factor_assays.html '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!

    52 min
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About

Welcome to Basics to Brilliance, the podcast created to supplement & bolster your knowledge of Haematology.Featuring a two way, non-didactic conversational-style Q&A between the SpR and SHO, this podcast will be your pocket companion no matter where you are.We aim to cover: - Malignant and non-malignant topics - Science/lab detail - UK guidelines, hallmark trials and how these translate into clinical practice - Future research directions - The whole syllabus for FRCPath part 1 All readily accessible and completely free of charge!For every budding haematologist out there, we hope this podcast aids you in your endeavours and fills you with interest and excitement for the brilliant world of Haematology. Warmest Regards,Dr. EverdenDr. FaseyDr. Jafri Disclaimer: This podcast is intended as a revision aid and should not be used for the medical management of patients. Guidelines in the initial episodes span 2023/2024. We aim to update our content in accordance with the most recent available guidelines when possible.  This podcast is CPD accredited by the Royal College of Pathologists UK 

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