Theme: Iron Overload Options A. Start subcutaneous desferrioxamine (DFO) B. Start deferasirox (DFX) C. Start deferiprone (DFP) D. Add deferiprone to desferrioxamine E. Combination: deferasirox + desferrioxamine F. Combination: deferiprone + deferasirox G. Continue current therapy and monitor H. Emergency IV continuous desferrioxamine infusion I. Venesection J. Reduce dose / avoid deferasirox due to renal impairment K. Start iron chelation (indication met) L. Intensify therapy

Theme: Iron overload Options: A. Calculate annual transfusional iron loading B. Ferritin monitoring every 1–3 months C. Baseline cardiac and liver MRI assessment D. MRI with sedation E. Routine interval MRI surveillance F. Annual LVEF assessment (echo or MRI) G. Liver MRI due to high liver iron burden H. Liver MRI triggered by rising ferritin trend I. Liver MRI (NTDT ferritin threshold >800 μg/L) J. Baseline MRI (NTRIA ferritin >1000 μg/L) K. Consider MRI (NTRIA ferritin >500

Theme: Diagnosis and Management of Congenital Dyserythropoietic Anaemia Options A. CDA type I (CDAN1 mutation) B. CDA type II (CDAN2 mutation) C. CDA type IIID. Iron deficiency anaemia E. β-thalassaemia major F. Myelodysplastic syndrome (erythroid subtype) G. Paroxysmal nocturnal haemoglobinuria H. Hereditary spherocytosis I. Aplastic anaemia J. CDA type II with indication for splenectomy Question 1 A male neonate born at 36 weeks is noted to have pallor and mild hepatospleno

Theme: Prevention and management of recurrent febrile and allergic transfusion reactions Options A. Trial of premedication with oral paracetamol before transfusion B. Trial of NSAIDs prior to transfusion (after risk assessment) C. No prophylaxis; investigate alternative causes (e.g. drugs/latex) D. Washed red cells/platelets for future transfusions E. Routine antihistamine prophylaxis F. Switch from apheresis platelets to pooled platelets (PAS) G. Solvent–detergent-treated pl

Theme: Recognition and Management of Transfusion Reactions Options A. Continue transfusion with paracetamol and close observation B. Slow transfusion and give antihistamine C. Stop transfusion and administer IM adrenaline immediately D. Stop transfusion and send unit for urgent bacterial culture E. Stop transfusion and perform full haemolytic reaction work-up F. Continue transfusion as hypotension likely due to haemorrhage G. Stop transfusion and arrange urgent chest X-ray H.

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Theme: Primary Prevention & Risk Stratification in aPL Carriers Options A. No pharmacological thromboprophylaxis; lifestyle advice only B. Start low-dose aspirin (LDA) C. Start hydroxychloroquine (HCQ) D. LDA + HCQ E. Thromboprophylaxis with LMWH in high-risk situation only F. Long-term anticoagulation with warfarin G. Avoid oestrogen-containing therapy; offer alternative contraception H. Start statin therapy I. Antenatal LMWH prophylaxis ± postnatal continuation J. No interv

Theme: Heparin monitoring and perioperative anticoagulation in APS/LA patients undergoing cardiac surgery Options (Each option may be used once, more than once, or not at all) A. Measure baseline ACT pre-operatively B. Use heparin anti-Xa monitoring during CPB C. Use standard ACT targets without adjustment D. Use patient-adjusted ACT based on baseline ACT E. Post-operative Prphylactic LMWH F. Post-operative thromboprophylaxis or treatment dose anticoagulation based on prior

Theme: CAPS in APS patients Options (Each option may be used once, more than once, or not at all) A. Start intravenous unfractionated heparin (UFH) infusion B. Monitor anticoagulation using anti-Xa levels rather than APTT C. Start high-dose corticosteroids D. Start IVIG E. Initiate plasma exchange F. Start rituximab G. Identify and treat triggering factor (e.g. infection, surgery) H. Multidisciplinary management including ICU + haematology I. Switch to warfarin once stable

Theme: Obstetric APS Options (Each option may be used once, more than once, or not at all) A. Aspirin alone throughout pregnancy B. Aspirin + prophylactic LMWH from positive pregnancy test C. Therapeutic-dose LMWH throughout pregnancy D. Switch from warfarin to LMWH immediately on confirmation of pregnancy E. Continue warfarin until second trimester F. Add hydroxychloroquine G. Add prednisolone or IVIG H. Refer to specialist obstetric–haematology centre I. Continue LMWH for

Theme: APS Options (Each option may be used once, more than once, or not at all) A. Increase warfarin target INR to 3.0–4.0 B. Add low-dose aspirin while maintaining INR 2.0–3.0 C. Switch from DOAC to warfarin D. Assess time in therapeutic range (TTR) and INR reliability E. Add hydroxychloroquine F. Refer to a specialist APS centre G. Add rituximab H. Switch to long-term therapeutic LMWH I. Continue current management without change J. Intensify cardiovascular risk manageme

Theme: APS Options (Each option may be used once, more than once, or not at all) A. Test full aPL panel (LA, aCL IgG/IgM, anti-β2GPI) now B. Do not test for aPL C. Repeat aPL testing after ≥12 weeks D. Defer LA testing due to anticoagulation interference E. Defer LA testing due to recent thrombosis/acute phase F. Perform DRVVT and LA-sensitive APTT G. Perform mixing study and phospholipid neutralisation step H. Start VKA (target INR 2.0–3.0) indefinitely I. Increase VKA inten

Theme: Inherited Bone Marrow Failure Syndrome Options (Each option may be used once, more than once, or not at all) A. FANCA B. FANCC C. DKC1 D. TERT E. RPS19 F. RPL11 G. SBDS H. ELANE I. MPL J. GATA2 K. SAMD9 L. MECOM Questions Q1 An 8-year-old boy presents with progressive pancytopenia, short stature, and radial ray abnormalities. He has café-au-lait pigmentation and renal anomalies. Bone marrow is hypocellular. Chromosomal breakage testing using diepoxybutane (DEB) is ma

Theme: Inherited bone marrow failure syndromes Pathophysiology OPTIONS (Each option may be used once, more than once, or not at all) A. Defective DNA interstrand crosslink repair leading to chromosomal instability B. Critically short telomeres causing stem cell exhaustion C. Haploinsufficiency of ribosomal proteins leading to p53 activation D. Endoplasmic reticulum stress impairing neutrophil maturation E. Defective thrombopoietin receptor signalling impairing megakaryopoie

Theme: Inherited bone marrow failure syndromes OPTIONS (Each option may be used once, more than once, or not at all) A. Fanconi anemia B. Dyskeratosis congenita C. Diamond–Blackfan anemia D. Shwachman–Diamond syndrome E. Severe congenital neutropenia F. Congenital amegakaryocytic thrombocytopenia G. GATA2 deficiency H. SAMD9/SAMD9 L disorder I. MECOM-associated syndrome Questions Q1 An 8-year-old boy is referred with progressive pancytopenia. He has a history of being sma

Theme: Waldenstrom OPTIONS (Each option may be used once, more than once, or not at all) A. Immediate plasmapheresis B. Pre-emptive plasmapheresis prior to rituximab C. Observation only D. Rituximab monotherapy E. DRC (dexamethasone–rituximab–cyclophosphamide) F. Bendamustine–rituximab G. BTK inhibitor H. Bortezomib-based regimen I. Treat as high-grade lymphoma J. Intrathecal/CNS-penetrating chemotherapy 1. A 65-year-old patient with WM develops: Painful acrocyanosis Haem

Theme: Waldenstrom OPTIONS (Each option may be used once, more than once, or not at all) A. Immediate plasmapheresis B. Pre-emptive plasmapheresis prior to rituximab C. Observation only D. Rituximab monotherapy E. DRC (dexamethasone–rituximab–cyclophosphamide) F. Bendamustine–rituximab G. BTK inhibitor H. Bortezomib-based regimen I. Treat as high-grade lymphoma (e.g. R-CHOP) J. Intrathecal + CNS-penetrating chemotherapy 1. A 72-year-old man presents with blurred vision, heada

Theme: Granulocyte Transfusion Options (Each option may be used once, more than once, or not at all) A. Start granulocyte transfusion immediately B. Do not give granulocytes – infection not refractory C. Not indicated – no expectation of neutrophil recovery D. Consider granulocytes after further optimisation E. Indicated due to neutrophil functional defect F. Not indicated – infection type inappropriate G. Needs MDT discussion with Microbiology and NHSBT about Prophylactic Gr

Theme: Granulocyte Transfusion Options (Each option may be used once, more than once, or not at all) A. Start granulocyte transfusion immediately B. Do not give granulocytes – infection not refractory C. Not indicated – no expectation of neutrophil recovery D. Consider granulocytes after further optimisation E. Indicated due to neutrophil functional defect F. Not indicated – infection type inappropriate G. Needs MDT discussion with Microbiology and NHSBT about Prophylactic Gr

Theme: Inherited thrombophilia Options A. Test for inherited thrombophilia B. Do not test – results will not influence management C. Test only if results will alter pregnancy management D. Test for specific deficiency (Protein C/S or Antithrombin) E. Advise against oestrogen-containing therapy and testing F. Defer testing until off anticoagulation G. Test asymptomatic first-degree relatives selectively H. Do not test – patient already in high-risk group I. Test for antiphosph