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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