Sickle Cell Disease

Question 1

Immunodeficiency in sickle cell disease-Pathogenetic mechanisms in the lungs

Answer 1

Infarction leads to a decrease in surfactants which leads to decreased bacterial preparation

Decreased macrophage function
Decreased polymorphonuclear leukocyte function

Question 2

Immunodeficiency in sickle cell disease-Pathogenetic mechanisms in the Spleen

Answer 2

Functional asplenia from 6months of Age
Autosplenectomy
Fibrosis due to Infarction

Question 3

Immunodeficiency in sickle cell disease-Pathogenetic mechanisms iron metabolism

Answer 3

Iron overload from destruction of RBCs
Increased iron overload from Exchange blood transfusions

Excess iron leads to;
Increased growth and multiplication of microbes
Increased iron will lead to Inhibition of macrophage function

Question 4

Immunodeficiency in sickle cell disease-Pathogenetic mechanisms in the complement System

Answer 4

Decreased synthesis of Propadine by spleen and polymorphonuclear leukocytes

Defective function of propadine
Propadine used in RBC clearance