PE26.4 | Hemolytic Anaemia — Summary & Reflection

KEY TAKEAWAYS

Haemolytic anaemia presents with the cardinal triad of anaemia + unconjugated jaundice + splenomegaly, confirmed by elevated reticulocytes, elevated LDH, reduced haptoglobin, and elevated indirect bilirubin. The peripheral smear is the most information-rich investigation after the CBC. Five major conditions: G6PD deficiency (X-linked recessive; oxidant-triggered; bite cells + Heinz bodies; enzyme assay after crisis resolves; trigger avoidance); thalassaemia major (autosomal recessive; presents >6 months; target cells + nucleated RBCs; transfuse to Hb ≥9–10 + chelate when ferritin >1000 µg/L); sickle cell disease (HbSS; vasoocclusion; sickle cells + Howell-Jolly bodies; hydroxyurea + penicillin prophylaxis); hereditary spherocytosis (mostly AD; spherocytes + elevated MCHC; osmotic fragility/EMA test; splenectomy after age 5–6); AIHA (acquired; DAT positive; warm type = steroids). Key distinguisher: DAT negative in all congenital conditions; DAT positive only in AIHA.

REFLECT

Consider the national burden: India births over 10,000 thalassaemia major children per year and has the world's largest SCD population outside Africa. Yet carrier screening before marriage and newborn screening remain inconsistently implemented. As a paediatrician, you will care for children whose condition could have been prevented with antenatal counselling — and you may be the first clinician to explain to parents what thalassaemia major or SCD means for their child's future. How would you explain to the parents of a newly diagnosed 8-month-old with thalassaemia major what the next 10 years of management will involve — transfusions every 3–4 weeks, monthly ferritin monitoring, chelation injections — in a way that is honest but not overwhelming? And if you were working in a tribal-area PHC in Odisha with a high SCD prevalence, what preventive interventions would you advocate for?