Clinical Cases Of Hemoglobin

Question 1

Sickle cell anemia

Answer 1

1) Decreased Hb in blood
2) Increase in serum total bilirubin. Direct is increased but indirect is increased more.
3) Increase in uric acid level.
4) Presence of HbS band in electrophoresis.
5) Peripheral smear shows presence of sickle cell RBCs

Sickle cell disease is autosomal recessive disease which occurs due to mutation in beta globin chain. Glutamic acid is replaced by valine at 6th position.

Sickling of RBCs is due to polymerisation of HbS. Valine has sticky ends to which attached sockets of alpha chains.

Question 2

Thalassemia major

Answer 2

Frontal bossing
Depressed cranial vault
Maxillary widening
Saddle nose
Hypochromic microcytic anemia.
Tear drop shaped RBCs

Thalassemia occurs due to unbalanced production and accumulation of alpha or beta globin chain.