Thalassemias MCQ — NEET PG Practice Question | NEETPGAI
Thalassemias
medium
microscope Pathology
Which is the most common cause of death in patients with β-thalassemia major in the modern era of transfusion therapy and iron chelation?
A. Acute leukemia
B. Cardiac arrhythmia secondary to iron overload
C. Hepatic cirrhosis and portal hypertension
D. Acute bacterial sepsis
Explanation
Leading Cause of Death in β-Thalassemia Major
Key Point
Cardiac arrhythmia and cardiomyopathy secondary to myocardial iron deposition are the most common causes of death in β-thalassemia major patients in the modern transfusion era.
Evolution of Mortality Causes Over Time
Table
Era
Primary Cause of Death
Secondary Causes
Pre-transfusion (1960s–70s)
Severe anemia, multi-organ failure
Infection, hemorrhage
Early transfusion (1980s–90s)
Hepatic cirrhosis, iron overload
Cardiac involvement emerging
Modern era (2000s–present)
Cardiac arrhythmia/cardiomyopathy
Sepsis, liver disease (if suboptimal chelation)
Mechanism of Cardiac Iron Overload
1.
Transfusional iron loading: Each unit of PRBC contains ~250 mg iron; patients receive 10–20 units/year
2.
Myocardial iron deposition: Iron accumulates in cardiomyocytes, causing oxidative damage via Fenton chemistry
Cardiomyopathy: Progressive systolic dysfunction and sudden cardiac death
High-YieldNEET PG
Cardiac iron overload is now the leading cause of death in well-transfused β-thalassemia patients because improved transfusion and chelation protocols have extended survival, unmasking chronic cardiac toxicity.
Clinical Pearl
T2* cardiac MRI is the gold standard for detecting myocardial iron and predicting arrhythmia risk; it should be performed annually in all β-thalassemia major patients.
Mnemonic
CARDIAC — Chelation Absent/Inadequate, ARrhythmia, Dilated cardiomyopathy, Iron overload, Arrhythmia, Cardiomyopathy, Death
Why Hepatic Cirrhosis Is No Longer #1
Although hepatic iron overload and cirrhosis remain significant complications, modern iron chelation (deferasirox, deferiprone, deferoxamine) has substantially reduced hepatic mortality. However, cardiac iron is more difficult to chelate and remains the leading cause.
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