SICKLE CELL ANEMIA

What is Sickle Cell?

 People who have Sickle
Cell have sickle shaped
red blood cells, which
causes complications
because the blood cells
are not able to reach
certain parts of the body.
Hb S
 Valine replaces glutamic, in the B
polypeptide chain.
 This alters the secondary and thirtiary 3
ry structures of B chain giving rise to Hb
S molecules which clump together,
causing the “sickling” of the cells.
 Pathogenesis
 There are 4 protein subunits of Hemoglobin A
A CARRIER FOR SICKLE CELL WILL
HAVE…
•An S mutation in
one copy of the
hemoglobin beta
gene.
•Half of the beta

subunits are
replaced with Beta
S.
•This person has the

Sickle Cell trait.

 SICKLE CELL DISEASE:
HBSS
•This results when
both copies of the
hemoglobin beta
gene have an S
mutation.
•All of this person’s

beta subunits are

replaced by S.
inheritance
Red blood cells Going through Vessels

Sickle cells tend to block the small

blood vessels
This gives rise to micro infarcts

Abnormal RBC being destroyed from

the circulation gives rise to anemia
Symptoms of Sickle Cell
 Anemia-
pallor,breathlessness,fatigue
 Jaundice
 Delayed growth and puberty
 Abdominal and bone/joint pain
 Greater risk for infection
 Adolescents and adults can
develop ulcers on their legs
 Chest pain
 Excessive thirst
 Poor eyesight, blindness
 Auto splenectomy-due to micro
infarcts in the spleen
 Crisis

 Acute chest syndrome

 Aplastic crisis
 Dactylitis – swelling of the hands and feet
 Painful crises: really painful episodes when blood
cells are blocked from going to certain parts of the
body – pain can occur anywhere, but it is usually in
the chest, arms, and legs
 Acute Enlarged spleen – sickle cells pool in the
spleen, causing abdominal pain
 Stroke

An x-ray of a hand
swollen from dactylitis
TREATMENT OPTIONS FOR
SICKLE CELL ANEMIA
There is no known cure for sickle cell anemia.

 Blood Transfusions
 Drug Treatment-hydroxyurea/penicillin
prophylaxis as spleen not functioning
 Blood and Marrow Stem Cell Transplantation
 Gene Therapy

These main treatment options for the painful crisis

involves analgesics and oral and intravenous fluids.
Thank you…