SICKLE CELL ANEMIA

Blood smear findings: Sickle cells; also helmet, burr, schisto-cytes, acanthocytes

Complications of sickling:

1. Hand-Foot syndrome
2. Splenic effects: Sequestration, progressive infarctions; lose ability to phagocytose encapsulated bacteria
3. Renal effects: Lose concentrating ability
4. "Painful crises"
5. "Acute chest syndrome"
6. Cerebrovascular Disease-About 10% of pts with SCA have a CVA by age 20

Sickle trait has been associated with splenic infarction, usually during strenuous physical exercise after a recent arrival at a high altitude. May also be linked to exercise-related rhabdomyolysis and exercise-associated sudden death.

Treatment (note--this list is by NO means complete--ER)

• Chronic transfusion, titrated to maintain HbS to < 30% of total Hb
  • Associated with RR 0.37 for painful crisis and RR 0.13 for acute chest syndrome (both sig.) in a randomized trial of 130 kids 2-16yo with SS anemia followed an avg. of 20mos (Stroke Prevention Trial--"STOP", J. Peds. 139:785, 2001--JW)
  • Also associated with reduction in CVA risk; transcranial doppler ultrasound can help identify pts at high risk for CVA for targeted treatment with chronic transfusion
  • Can cause iron overload
• Hydroxyurea--Reduces incidence of vaso-occlusive complications of SS anemia