Authors :
Adrita Das; Sweta Yadav; Saptarshi Bhattacharyya; Arpita Patnaik; Sushree swagatika subhadarsini; Dr. Gopal Krishna Purohit; Dr. llalli Smruti Sahu
Volume/Issue :
Volume 7 - 2022, Issue 2 - February
Google Scholar :
http://bitly.ws/gu88
Scribd :
https://bit.ly/3BDKEbT
DOI :
https://doi.org/10.5281/zenodo.6201218
Abstract :
Sickle cell disease (SCD) is usually caused due to
the alteration of the beta-globin subunit in the primary
adult haemoglobin (HbA). When both the beta-globin
subunits get mutated, i.e., in the heterozygous condition, the
HbS polymerizes upon deoxygenation, resulting in the
erythrocytes acquiring sickle or crescent shape, known as
sickling. During the initial process, the acquired
erythrocytes often sway between the circular and sickle
shapes when oxygen pressure is lower. However, the
situation becomes critical if the change becomes irreversible
resulting in permanent sickling of the erythrocytes,
worsening the threat of hemolysis and vascular damage.
The most common form of the disease is sickle cell anemia,
where the patient is susceptible to hemolysis. Every year,
thousands of cases are being reported, the majority being
from Sub-Saharan Africa. Despite the genetic similarity at
the site of mutation, not all patients get equally affected. If
the sickle cell disease is left untreated for long, it causes
neurological complications, pulmonary hypertension,
cardiac disease, renal complications etc. Various methods
have been developed for the treatment of the disease. This
review briefly discusses sickle cell disease, its
pathophysiological aspects, its causes, effects and
treatment.
Keywords :
Sickle Cell Anemia, Pathophysiology, Hemolysis, Thalassemia, Mutation.
Sickle cell disease (SCD) is usually caused due to
the alteration of the beta-globin subunit in the primary
adult haemoglobin (HbA). When both the beta-globin
subunits get mutated, i.e., in the heterozygous condition, the
HbS polymerizes upon deoxygenation, resulting in the
erythrocytes acquiring sickle or crescent shape, known as
sickling. During the initial process, the acquired
erythrocytes often sway between the circular and sickle
shapes when oxygen pressure is lower. However, the
situation becomes critical if the change becomes irreversible
resulting in permanent sickling of the erythrocytes,
worsening the threat of hemolysis and vascular damage.
The most common form of the disease is sickle cell anemia,
where the patient is susceptible to hemolysis. Every year,
thousands of cases are being reported, the majority being
from Sub-Saharan Africa. Despite the genetic similarity at
the site of mutation, not all patients get equally affected. If
the sickle cell disease is left untreated for long, it causes
neurological complications, pulmonary hypertension,
cardiac disease, renal complications etc. Various methods
have been developed for the treatment of the disease. This
review briefly discusses sickle cell disease, its
pathophysiological aspects, its causes, effects and
treatment.
Keywords :
Sickle Cell Anemia, Pathophysiology, Hemolysis, Thalassemia, Mutation.
