Initially the spleen gets enlarged as it is performing more functions. Leads to decrease in spleen size - Auto splenectomy.Sequestration crisis: All sickle cells are sequestered into the spleen.Hemolytic crisis: Associated with Ebstein Bar Virus.Aplastic crisis: Associated with parvovirus B19 infection.Fish mouth valve is seen in - Rheumatic Heart Disease (RHD).Lungs - results in acute chest syndrome.Vaso-occlusive crisis in different organs.Jaundice - RBCs are broken therefore increases bilirubin.Splenomegaly - It is an extravascular hemolysis and broken by spleen.If these get stuck in the narrow blood vessels - results in vaso-occlusive crisis. They stick to each other and forms a mass. These sickle shaped cells are very sticky. When there is repeated reversible sickling it leads to irreversible sickling. When there is enough oxygen and proper PH, the sickle shaped cells are converted to normal again, called reversible sickling. In the absence of oxygen and less pH The cells convert to sickle shape. If it contains one normal and one harmful gene - heterozygous.β thal and Sickle cell genes are present.Like HbβS - Here both the genes are harmful.Sickle cell anemia is autosomal resistance disease.So as there is less hemoglobin, sickling is less.Fetal hemoglobin has more affinity to oxygen.Normal Cell converts to Sickle cell when: So, it is a missense point mutation.Īt β 6 position, if glutamic acid changes to lysine. When changed to valine the HbA changes to sickled hemoglobin (HbS). ![]() When glutamic acid is present - patients have HbA.
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