Haem: Haemophilia A: Free MSRA Podcast

🎧 Haemophilia A – Deep Dive for MSRAIn this high-yield episode, we explore Haemophilia A, a key genetic bleeding disorder for the MSRA. Learn how to recognise, diagnose, and manage this condition with clarity and confidence. From Factor VIII deficiency to treatment options and complications, we cover all the essentials in one focused revision session.🧬 What is Haemophilia A?A genetic bleeding disorder due to Factor VIII deficiency → poor clot formation → prolonged bleeding.🧠 Mnemonic:"A is for APTT ↑ in haemophilia A"• Inherited (X-linked recessive)• F8 gene mutation on Xq28• Affects males, females = carriers📊 Severity Levels:• Severe: <1% Factor VIII → spontaneous bleeds• Moderate: 1–5% → bleeds with mild trauma• Mild: >5% → bleeds with surgery/injury🩸 Symptoms:• Easy bruising• Joint bleeds (knees, ankles, elbows)• Prolonged bleeding post-injury/surgery• Bleeding after heel prick/circumcision• Muscle, GI, or intracranial bleeds in severe cases🧪 Diagnosis:• Low Factor VIII activity• ↑ APTT (normal PT, bleeding time, platelets)• Genetic testing for F8 mutation• Newborns: cord blood APTT & Factor VIII🧠 Differentials Mnemonic:“Very Bad Acquired Cold, Very Funny Platelet Vessel”V: Von WillebrandB: Haemophilia B (Factor IX)A: Acquired coag disordersC: Haemophilia CV: Vitamin K deficiencyF: Fibrinogen/FibrinolysisP: Platelet issuesV: Vessel fragility💉 Treatment:1. Factor VIII ReplacementProphylactic (2–3x/week or extended half-life)On-demand for bleeds2. Desmopressin (DDAVP)Mild cases – stimulates endogenous Factor VIII3. Tranexamic AcidUseful for mucosal bleeds4. PhysiotherapyPrevents joint damage post-bleed❌ Avoid:• NSAIDs (e.g. ibuprofen, aspirin)• IM injections (bleeding risk)• Unplanned physical trauma🧠 Emergency Mnemonic: "ABC + Factor VIII"Airway, Breathing, Circulation… + urgent Factor VIII if life-threatening bleed🩻 Investigations:• FBC: check for anaemia• Imaging: MRI or US for joint bleeds• CT: for suspected intracranial haemorrhage📉 Complications:• Haemarthrosis → joint damage (arthropathy)• Inhibitor development (anti-Factor VIII Abs)• Rare: life-threatening bleeds or historical infection risk (now low)👶 Pregnancy & Carriers:• Female carriers: 50% chance of passing gene to sons• Multidisciplinary delivery planning• Test baby boys at birth if at risk📈 Prognosis:Greatly improved with prophylaxis 💉Good QoL possible with education, adherence & early treatment🧪 Key MSRA Resources:📝 Revision Notes:https://www.passthemsra.com/topic/haemophilia-a-revision-notes/🧠 Flashcards:https://www.passthemsra.com/topic/haemophilia-a-flashcards/❓ Q&A Notes:https://www.passthemsra.com/topic/haemophilia-a-accordion-qa-notes/🔥 Rapid Quiz:https://www.passthemsra.com/topic/haemophilia-a-rapid-quiz/🧪 Practice Quiz:https://www.passthemsra.com/quizzes/haemophilia-a/#MSRA #HaemophiliaA #MSRARevision #MSRAQuiz #MSRAFlashcards #HaematologyMSRA #FactorVIII #MSRAExam #MSRANotes #PasstheMSRA #TheDeepDive