All the following are seen in thalassemia major except:
Correct Answer: Low iron content in blood
Description: Ans: c (Low iron content in blood)Serum iron content is increased in thalassaemia major and not decreased.THALASSAEMIA MAJOR / COOLY'S ANAEMIAAge is usually 3-6 monthsRegular, monthly blood transfusion dependentClinical Features* Mainly due to extramedullary erythropoiesis in facial and skull bones -> haemolytic facies* HepatosplenomegalyLab Findings* Peripheral smear- hypochromic microcytic anaemia with bizarre fragmented poikilocytes* Intra erythrocytic inclusions - Heinz bodies Heinz bodies are precipitated extra alpha chain* Hb F increased* TIBC normal* Osmotic fragility is reduced* X ray skull - hair on end appearance.Complications 1) Hypersplenism 2) Haemosiderosis 3) Endocrine complications4) Cardiomyopathy - cause of death# Diabetes mellitus# Hypothyroidism# Delayed puberty5) Hyperpigmentation due to melanin deposition6) Growth retardationTreatmentRegular packed cell transfusionBone marrow transfusion is curative
Category:
Pediatrics
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