Sickle cell disease

1. Sickle Cell Disease

2. Definition :
 Is one of the Group of inherited disorders known as sickle cell disease .
 It affects the shape of RBCs which carry oxygen to all parts of the body .
 Normal RBCs are usually round and flexible , so they move easily through
blood vessel , but Sickle cell anemia become rigid and Sticky which can
slow and block blood flow

3. Symptoms :
 Anemia
Sickle cells break apart easily and die typically in 10-20 days leaving a shortage
of RBCs ( anemia ) [ normal RBCs need 120 days to be Replaced ] .
 Episodes of pain :
Pain develops when sickle shaped RBCs flow through tiny blood vessels to abdomen
, chest and joints . Pain varies in intensity and can last for few hours to few days .
 Swelling of hands and feet ,
Caused by sickle shaped RBCs blocking blood circulation in the hands and feets .
 Frequent infections :
Sickle shaped can damage the spleen
 Vision Problems :
Sickle shaped RBCs can plug blood vessels that supply eyes .

4. Complications :
 Stroke : Sickle RBCs can block blood flow to areas of brain , it can cause
symptoms of Seizures , weakness , numbness of the arms and legs

5.  Pulmonary hypertension :
Sickled RBCs can cause high blood
pressure

6. Organ Damage :
 Sickle cells that block blood flow to organs
deprive the affected organs of blood and
oxygen.
 This lack of oxygen rich blood can damage
nerves and organs including kidneys , liver
and spleen .

7. Deep vein thrombosis :
Sickling of red Cells can
cause blood clots ,
increasing the risk of a clot
lodging in a deep vein (deep
vein thrombosis ) or a lung
(pulmonary embolism ) .

8. Avascular Necrosis [AVN ] (Death of
bone tissue ) :-
 Sickled RBCs can block blood flow in
blood vessels that provide blood to
bones , when bone doesn't get enough
oxygen , the bone tissue can die or
osteoarthritis may occur . The joint
can narrow and the bone can collapse .
 The most common location of AVN is
Hip joint
 AVN may occur without any symptoms ,
but as AVN progresses , it can result in
mild to severe joint pain .

9. Sickled RBCs can affect
Cardiovascular and cause :
- Cardiomegaly
- Left ventricular dysfunction
- Diastolic dysfunction

10. Sickled RBCs can affect
abdomen and cause :
- Splenic infarction
- Splenomegaly
- Hepatic cirrhosis (chronic liver
disease processes that cause
hepatocellular necrosis )

11.  H-Shaped Vertebrae : are a
characteristic finding of
sharply delimited central
endplate depression , results
from microvascular endplate
infarction

12. Bony Sclerosis