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  Sickle cell anaemia

Sickle cell anaemia:

Sickle cell anaemia is the genetic syndrome caused by autosomal mutant allele Hbs. In homozygous situation (Hbs Hbs), it causes the making of abnormal haemoglobin termed as haemoglobin S. The usual haemolobin is designated as HbA (HbAHbA). Sickle cell persons with the genotype HbsHbs suffer from a fatal haemolytic anaemia. The patient expires due to damaged kidney, heart, spleen and brain as an outcome of clogged blood vessels or vascular obstruction. The person with heterozygous genotype HbA Hbs is state to be carriers and they survive.

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Causes of Sickle cell anaemia:

Insufficient production of red cells:

  • by insufficient "raw materials"
  • Iron deficiency
  • Vitamin B12 deficiency
  • Folic acid deficiency
  • by impaired function of bone marrow factory
  • Anaemia of chronic disease
  • Bone marrow destroyed or damaged (i.e., aplastic anemia)
  • Bone marrow substituted by foreign or abnormal cells (i.e., bone marrow replacement anemia)


Excessive loss of red cells:

1) by external blood loss (i.e., hemorrhage)
2) by shortened survival of red cells in the circulation
3) Defective red cells (i.e., hereditary hemolytic anemia)

  • Abnormal red cell shape
  • Abnormal hemoglobin inside red cells
  • Defective hemoglobin synthesis inside red cells
  • Deficient red cell enzymes

4) "Hostile environment"

  • Anti-red cell antibodies
  • Mechanical trauma to circulating red cells

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