Hemolytic Anemias MCQ — NEET PG Practice Question | NEETPGAI
Hemolytic Anemias
easy
microscope Pathology
A 28-year-old woman of Indian origin presents with jaundice, dark urine, and splenomegaly. Blood film shows spherocytes and polychromasia. Osmotic fragility test is positive. What is the most common cause of hereditary hemolytic anemia in this patient?
A. Glucose-6-phosphate dehydrogenase deficiency
B. Hereditary elliptocytosis
C. Hereditary spherocytosis
D. Pyruvate kinase deficiency
Explanation
Most Common Hereditary Hemolytic Anemia
Key Point
Hereditary spherocytosis (HS) is the most common hereditary hemolytic anemia in Caucasian and Indian populations, accounting for approximately 50% of all hereditary hemolytic anemias in these regions.
Pathophysiology
HS results from mutations in genes encoding red cell membrane proteins (spectrin, ankyrin, band 3, or protein 4.2). This leads to:
1.
Loss of membrane surface area
2.
Formation of spherical RBCs (spherocytes)
3.
Osmotic fragility and splenic sequestration
Diagnostic Features
Table
Feature
Finding
Blood film
Spherocytes, polychromasia
Osmotic fragility
Positive (RBCs lyse at higher saline concentrations)
Indirect bilirubin
Elevated
Reticulocyte count
Elevated (>2%)
LDH
Elevated
Haptoglobin
Decreased
Clinical Presentation
Jaundice (unconjugated hyperbilirubinemia)
Dark urine (due to hemoglobinuria)
Splenomegaly (from RBC sequestration)
Gallstones (from chronic hemolysis)
Aplastic crisis (from parvovirus B19 infection)
High-YieldNEET PG
The combination of spherocytes + positive osmotic fragility test + family history is pathognomonic for HS.
Clinical Pearl
Splenectomy is curative in 70–80% of HS patients, as the spleen is the primary site of spherocyte destruction.
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