Sickle cell disease: Clinical

Early presentation:

• Hemolytic anemia

  • Starts in 1st few months of life

  • Occurs gradually as Hgb F replaced by Hgb SS

• Dactylitis

  • Painful swelling of extremities

  • Infarcts of distal small bones of hands/feet

Vaso-occlusive episodes

• Extremely painful

• Can affect multiple organ systems

• Brain (CVA)

• Lung (Acute chest syndrome)

• Spleen (autoinfarction)

• Kidneys (fibrosis/hyposthenuria/papillary necrosis)

• GU (priapism)

• Bone (AVN)

Splenic sequestration

• Sickling in spleen causes obstruction

• Cardiovascular collapse may occur

• Transfusion may be required

Aplastic crisis

• Rapid drop in Hgb level

• Associated with parvovirus B19 infection

Autosplenectomy

• Typically by 5 yrs of age

• increased risk of encapsulated organism infection

• sepsis, meningitis, osteomyelitis (Salmonella)

• (The liver also may infarct and progress to failure with time)

Hemolysis

• Chronic & well tolerated

• May lead to gallstone formation