Clinical manifestations : Sickle Cell Disease

Clinical manifestations

  • Typical patient is asymptomatic except during sickling episodes
  • Symptoms may include
    • Pain from tissue hypoxia and damage
    • Pallor of mucous membranes
    • Jaundice from hemolysis
      • Prone to gallstones (cholelithiasis)

Complications

Body organ

Complications

Brain

Thrombosis or hemorrhage causing paralysis, sensory deficits, death

Eye

Hemorrhage

Retinal detachment

Blindness

Retinopathy

Lung

Acute chest syndrome

Pulmonary hypertension

Pneumonia

Heart

Heart failure

Liver and gallbladder

Hepatomegaly

Gallstones

Spleen

Splenic atrophy (autosplenectomy)

Kidney

Hematuria

Renal failure

Bones and joints

Hand-foot syndrome

Osteonecrosis

Penis

Priapism

Skin

Stasis ulcers of hands, ankles, and feet

  • Infection is a major cause of morbidity and mortality
    • Function of spleen becomes compromised from sickled RBCs
      • Autosplenectomy is a result of scarring
    • Pneumococcal pneumonia most common
    • Severe infections can cause aplastic crisis
      • Can lead to shutdown of RBC production
  • Acute chest syndrome
    • Pulmonary complications that include pneumonia, tissue infarction, and fat embolism
    • Characterized by fever, chest pain, cough, pulmonary infiltrates, and dyspnea
    • Leads to multiple serious complications

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