Review Question - QID 104644

This patient most likely has a stroke (given his sudden onset facial asymmetry and weakness) as a result of his sickle cell disease and sludging of sickle cells causing microvascular occlusion. Treatment with exchange transfusion is the preferred treatment.

Sickle cell anemia is an autosomal recessive disorder that causes abnormal hemoglobin. HbS can sickle in the setting of stress, infection, or dehydration causing acute pain crises, priapism, acute chest syndrome, and stroke. The prevalence is highest in African-Americans. The treatment of an acute pain crisis is to rule out any infections, administer IV fluids, and administer analgesics. If patients have a life-threatening diagnosis such as acute chest syndrome, stroke, or priapism, exchange transfusion is indicated.

Incorrect Answers:
Answer 1: Alteplase is a thrombolytic which could be given in an acute ischemic stroke. If this patient was having a stroke secondary to a thrombus, then it could be appropriate in the setting of a head CT that does not show a bleed. In this patient, it is highly unlikely he is having an acute ischemic stroke secondary to a thrombus given his young age and his history of only sickle cell disease.

Answer 3: Heparin is an anticoagulant that can be used for a pulmonary embolism or a DVT. It is generally not the preferred treatment for an acute ischemic stroke or for crises in sickle cell disease.

Answer 4: Hydroxyurea is a drug the patient should chronically be on as it increases HbY formation and is useful for this patient's anemia. It is not indicated for acute complications of sickle cell disease.

Answer 5: Warfarin is an anticoagulant that patients may be started on for chronic anticoagulation for conditions such as atrial fibrillation, a DVT, or a pulmonary embolism (after initially being started on heparin).

Bullet Summary:
Stroke in sickle cell disease is best treated with exchange transfusion.