Sickle cell anemia

Alternative names:

anemia - sickle cell; Hemoglobin SS disease (Hb SS); Sickle cell disease.

Symptoms:

• family history of sickle cell anemia
• fatigue
• breathlessness
• cough
• rapid heart rate
• delayed growth and puberty
• susceptibility to infections
• ulcers on the lower legs (in adolescents and adults)
• jaundice
• bone pain
• attacks of abdominal pain
• weakness
• joint pain
• fever
• vomiting

Additional symptoms that may be associated with this disease:

• bloody urine (hematuria)
• excessive urination, excessive volume
• thirst, excessive
• · Unwanted painful erection (priapism; this occurs in 10-40% of men with the disease)
• chest pain
• Poor eyesight/blindness
• decreased fertility

Signs and tests:

Common signs include:

• Paleness
• Yellow eyes/skin
• Variable tenderness, depending on the symptoms
• Growth retardation

Tests commonly performed to diagnose and monitor patients with sickle cell anemia include:

• CBC
• hemoglobin S screening test
• hemoglobin electrophoresis
• sickle cell test

Patients with sickle cell may have abnormal results on certain tests, as follows:

• urinary casts or blood in the urine
• peripheral smear displaying irreversible sickle cells (ISC)
• hemoglobin; serum
• elevated ESR
• elevated bilirubin
• High white blood cell count (This can predict more complications)
• elevated serum potassium
• elevated serum creatinine
• chest x-ray may display signs of infection, both during an actual infection and during acute chest syndrome.
• blood oxygen saturation may be decreased.
• EKG can show evidence of ischemia (poor blood flow) to the heart
• CT scan or MRI can display strokes

Updated Date: 05/08/00

Updated by: Bradley G. Somer, MD, Division of Hematology-Oncology, Hospital University of Pennsylvania, Verimed Health Network