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
- Function of spleen becomes compromised from sickled RBCs
- 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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