# Haemolytic Anaemias and Thalassaemia > Haemolytic anaemias and thalassaemia for FMGE Medicine: warm AIHA steroids, G6PD drug list, transfusion-chelation protocols and Indian screening. - Canonical URL: https://prepelephant.com/topics/fmge/medicine/haemolytic-anaemias-and-thalassaemia - Exam / course: FMGE · Subject: Medicine - Publisher: PrepElephant (https://prepelephant.com) — Prepared and reviewed by the PrepElephant Academic Review Team - First published: 2026-10-02 - Last updated: 2026-10-02 - How to cite: "Haemolytic Anaemias and Thalassaemia", PrepElephant, https://prepelephant.com/topics/fmge/medicine/haemolytic-anaemias-and-thalassaemia ## Direct answer Transfusion-dependent beta thalassaemia major is managed by transfusions holding pre-transfusion haemoglobin at 9-10.5 g/dL, lifelong iron chelation — deferasirox 20-40 mg/kg daily orally, or subcutaneous deferoxamine 40-50 mg/kg overnight, 5-6 nights weekly — and surveillance of endocrine, cardiac and hepatic iron burden; haematopoietic stem cell transplant is the only cure, offered free to eligible Indian children under the Thalassemia Bal Sewa Yojana. Warm autoimmune haemolytic anaemia responds to prednisolone 1 mg/kg with rituximab or splenectomy for refractory disease; G6PD deficiency is treated by withdrawing the trigger (primaquine, dapsone, sulfa drugs, nitrofurantoin, fava beans); hereditary spherocytosis is confirmed by EMA binding and cured functionally by splenectomy. India carries a 3.5-4 percent beta-thalassaemia trait prevalence, making carrier screening and prenatal diagnosis the prevention backbone. ## What you must remember - **Haemolysis fingerprint:** reticulocytosis, raised indirect bilirubin and lactate dehydrogenase, low haptoglobin, and the blood film's spherocytes, fragmented cells or sickle forms pointing to the mechanism. - **Warm AIHA:** IgG-mediated, spherocytes on film, positive direct antiglobulin (Coombs) test; treat with prednisolone 1 mg/kg tapering slowly, then rituximab, splenectomy or ciclosporin for refractory disease; cold AIHA (IgM, Mycoplasma or infectious mononucleosis) responds poorly to steroids. - **G6PD deficiency:** X-linked, episodic haemolysis with Heinz bodies and bite cells after oxidant drugs — primaquine, dapsone, cotrimoxazole, nitrofurantoin, rasburicase — or fava beans and infection; avoid the trigger, transfuse only for profound anaemia. - **Hereditary spherocytosis:** EMA binding test (osmotic fragility is the classical alternative); folate lifelong; splenectomy for severe disease, only after vaccination against pneumococcus, meningococcus and Haemophilus influenzae type b, with penicillin prophylaxis. - **Thalassaemia major basics:** presents at 6-12 months with severe microcytic anaemia (high iron, unlike iron deficiency), haemoglobin electrophoresis showing raised HbF and HbA2 in trait; transfusion plus chelation prevents the facies, growth failure and cardiac death of unchelated disease. - **Chelation targets:** serum ferritin trends plus MRI T2* of liver and heart — cardiac T2* below 20 ms signals myocardial iron and imminent risk, intensified chelation can reverse it. - **Transplant and prevention:** matched-sibling stem cell transplant cures; India's Thalassemia Bal Sewa Yojana (Ministry of Health and Family Welfare with Coal India CSR support) funds transplants for eligible thalassaemia major children, and carrier screening targets high-prevalence communities. - **Sickle cell corner:** central-Indian tribal belt prevalence; hydroxyurea raises HbF and reduces crises; vaso-occlusive crisis managed with hydration and analgesia. ## How to work through a child with severe anaemia A nine-month-old brought from a thalassaemia-major family is pale and failing to thrive; haemoglobin is 6.5 g/dL, MCV 62 fL, ferritin high, electrophoresis showing markedly raised fetal haemoglobin. The diagnosis is beta thalassaemia major: regular transfusions every 2-4 weeks keeping pre-transfusion haemoglobin at 9-10.5 g/dL — enough for growth and to suppress the extramedullary haematopoiesis that carved the bony facies of the untreated era. Each unit delivers iron the body cannot excrete, so chelation begins after about 10-15 transfusions: oral deferasirox each morning, with ferritin trends and, from school age, annual MRI T2* of liver and heart, because unchelated death is cardiac — arrhythmia and failure from myocardial iron. Her parents are counselled on two family-level decisions: a matched-sibling transplant referral under the Thalassemia Bal Sewa Yojana if a donor exists, and genetic counselling — two carriers carry a one-in-four risk per pregnancy, so prenatal diagnosis by chorionic villous sampling at 10-12 weeks is offered. Next door, a young man started on primaquine for vivax malaria returns jaundiced with dark urine — G6PD deficiency unmasked; treatment is withdrawal of the trigger, folate support and transfusion only if profound. ## How the FMGE frames it The exam runs haemolysis as a two-step: first the laboratory fingerprint (raised reticulocytes, indirect bilirubin, LDH, low haptoglobin), then the discriminator — a positive direct Coombs means autoimmune; a negative sends you down the membrane-enzyme-haemoglobin chain. The G6PD drug list is quoted verbatim as options. Thalassaemia questions contrast trait (microcytosis with high iron and HbA2) against iron deficiency (low ferritin) — the most repeated differentiator. Numbers NBE expects: pre-transfusion haemoglobin 9-10.5, deferasirox 20-40 mg/kg, cardiac T2* thresholds. The Indian programme layer — trait prevalence near 4 percent, prenatal screening, and the government-funded transplant scheme — supplies community-medicine crossover one-liners. ## Frequently asked questions ### What haemoglobin target is maintained in thalassaemia major? Pre-transfusion haemoglobin of 9-10.5 g/dL through regular transfusion, enabling normal growth and suppressing extramedullary haematopoiesis. ### How is iron overload chelated in thalassaemia? Oral deferasirox 20-40 mg/kg daily, or subcutaneous deferoxamine 40-50 mg/kg overnight infusions 5-6 nights weekly, monitored with ferritin and MRI T2* of liver and heart. ### Which drugs must G6PD-deficient patients avoid? Primaquine, dapsone, cotrimoxazole, nitrofurantoin, rasburicase and other oxidants, plus fava beans — withdrawal of the trigger is the treatment. ### How is hereditary spherocytosis confirmed and cured functionally? Eosin-5-maleimide binding test (or osmotic fragility) confirms it; splenectomy removes the destruction site, preceded by vaccination against pneumococcus, meningococcus and Hib. ### What government support exists for thalassaemia major children in India? The Thalassemia Bal Sewa Yojana funds bone marrow transplantation for eligible children with thalassaemia major and aplastic anaemia at empanelled centres.