Question

How does sickle cell anemia affect different body systems like the circulatory system, respiratory system, and digestive system?

Answer 1

Answer with discussion and explanation :

Sickle cell anemia is a hemoglobinopathy due to inherited mutation in beta-globin chain. It is caused by point mutation at sixth position causing substitution of valine residue for glutamic acid resulting in sickle hemoglobin. When deoxygenated, aggregated sickle hemoglobin assemble and polymerise producing a distorted sickle cell RBC.

Irreversible sickle cells are responsible for hemolytic anemia whereas reversible sickle cells are responsible for vasoocclusive signs and symptoms. Vasooclusive signs and symptoms are:

1) Painful bone crisis - Very difficult to differentiate from osteomyelitis. Frequently involves hand and feet.

2) Spleen - Spleen gets infarcted before adulthood. Also termed as autosplenectomy.

3) Aplastic crisis - Infection by Parvovirus B19 causes aplastic crisis.

4) Acute chest syndrome - Condition characterised by fever, shortness of breath, chest pain due to sickling in pulmonary vascular beds.

5) Acute abdomen - Sickling in mesenteric and portal blood system along with presence of Veilonella bacteria causes pain abdomen.

6) Cardiovascular system - Vasoocclusive phenomenon may also result in myocardial infarction and peripheral vascular disease and gangren.