Haem: Myelofibrosis: Free MSRA Podcast

🎧 Myelofibrosis – Bone Marrow Fibrosis Demystified for the MSRA 🩸🧬Welcome to the Deep Dive! In this episode, we unpack Myelofibrosis (MF) — a rare but essential myeloproliferative neoplasm (MPN) to understand for the MSRA exam and clinical practice. Whether it's primary MF or secondary to PV/ET, we break it all down — from causes to complications.🧠 What You’ll Learn📌 Definition• Chronic bone marrow disorder where fibrosis disrupts normal blood cell production• Can be primary or secondary to polycythaemia vera or essential thrombocythaemia📌 Pathophysiology• Mutations in JAK2 (~50%), CALR, or MPL• Leads to abnormal stem cell proliferation, marrow fibrosis, and extramedullary haematopoiesis (in liver/spleen)📌 Risk Factors• Older age, male sex, prior chemo/radiation, other MPNs• Higher in white individuals and Ashkenazi Jews📌 Clinical Features (Mnemonic: FWEB9)• Fatigue• Weight loss• Enlarged spleen (splenomegaly)• Bone pain• 9 = 9pm (night sweats, constitutional symptoms)Also: anaemia, early satiety, infections, petechiae, bruising, portal HTN, gout📌 Investigations🔬 Bloods: Anaemia, thrombocytopenia/-cytosis, leucoerythroblastic picture, teardrop cells, ↑LDH🧬 Genetic: JAK2/CALR/MPL mutations; exclude BCR-ABL1; cytogenetics for prognosis🦴 Bone marrow biopsy: Fibrosis grading, megakaryocyte abnormalities🖥️ Imaging: USS/MRI for spleen; CT if extramedullary haematopoiesis suspected📌 WHO Diagnostic Criteria (2008)• Bone marrow fibrosis + clonal marker or reactive fibrosis excluded• Plus ≥2 of: anaemia, splenomegaly, constitutional symptoms, ↑LDH, teardrop cells💊 Management✅ Curative: Allogeneic stem cell transplant (allo-SCT) – high risk, for younger patients📉 Symptom control:• JAK inhibitors (Ruxolitinib) – shrink spleen, improve QOL• Hydroxycarbamide – for raised counts• Transfusions ± EPO – for anaemia• Splenectomy or radiotherapy – if splenomegaly refractory🛡️ Supportive: Gout prophylaxis, infection surveillance, nutrition, fatigue support📈 Prognosis• IPSS scoring: age, Hb, WCC, blasts, symptoms → risk stratification• Risk of AML transformation: 10–20%• Median survival: varies from 2 to >10 years⚠️ Complications• Anaemia & pancytopenia• AML transformation• Portal hypertension• Extramedullary haematopoiesis complications• Gout, infections, bleeding• Splenic infarction or rupture• Bone pain, osteosclerosis📚 Memory Aids• FWEB9 – Fatigue, Weight loss, Enlarged spleen, Bone pain, 9pm = Night sweats• Teardrop cells = marrow stress• Only allo-SCT is curative📝 MSRA Revision Resources• 📘 Revision Notes: https://www.passthemsra.com/topic/myelofibrosis-revision-notes/• 🃏 Flashcards: https://www.passthemsra.com/topic/myelofibrosis-flashcards/• 📂 Accordion Q&A: https://www.passthemsra.com/topic/myelofibrosis-accordion-qa-notes/• 🎯 Rapid Quiz: https://www.passthemsra.com/topic/myelofibrosis-rapid-quiz/• 🧪 Practice Quiz: https://www.passthemsra.com/quizzes/myelofibrosis/🌐 More Platformshttps://www.passthemsra.comhttps://www.freemsra.com#MSRA #Myelofibrosis #HaematologyForMSRA #MSRARevisionNotes #MSRAFlashcards #PassTheMSRA #JAK2 #CALR #Ruxolitinib #MPN #MSRAQuiz #BoneMarrow #AlloSCT #TeardropCells #MyeloproliferativeNeoplasm #FreeMSRA #MSRAPodcast