Sickle Cell Anemia

Question 1

What is abnormal in sickle cell anemia?

Answer 1

Hemoglobin; abnormal Hgb S alleles; sickle cell disease has two abnormal and sickle cell trait has one abnormal allele

Question 2

What is important to be aware of in athletes with sickle cell disease?

Answer 2

Increased risk of sudden exertional collapse; responds to oxygen and IV hydration

Question 3

What are the orthopedic manifestations of sickle cell disease?

Answer 3

1) sickle cell crisis (see below)
2) osteomyelitis
3) septic arthritis
4) osteonecrosis of femoral and humeral heads
5) bone infarcts
6) growth retardation / skeletal immaturity
7) dactylitis (acute hand / foot swelling)
8) biconcave vertebrae

Question 4

What is the most common organism involved with osteomyelitis in sickle cell patients?

Answer 4

Staph aureus; there is increased risk of salmonella in sickle patients

Question 5

What imaging modality can help distinguish bone infarct from osteomyelitis?

Answer 5

Radionuclide bone scan and bone marrow scan:

osteomyelitis: normal marrow uptake, abnormal bone scan
infarct: decreased marrow uptake, abnormal bone scan