hematology and oncology
Sickle Cell Disease
Symptoms and Signs of Sickle Cell Disease
Most symptoms occur only in patients who are homozygous and result from Anemia Vaso-occlusive events resulting in tissue ischemia and infarction Anemia is usually severe but varies among patients and is usually compensated; mild Jaundice and pallor are common. Hepatosplenomegaly is common in children, but because of repeated infarctions and subsequent fibrosis (autosplenectomy), the spleen in adults is commonly atrophied. Cardiomegaly and systolic ejection (flow) murmurs are common. Cholelithiasis and chronic punched-out skin ulcers around the ankles are common. Painful vaso-occlusive crisiscauses severe pain in long bones, the hands and feet, back, and joints. Hip pain may result from avascular necrosis of the femoral head. Severe abdominal pain, which may be due to hepatic vein thrombosis, may develop with or without vomiting and is usually accompanied by back and joint pain. Acute chest syndromeis characterized by sudden onset of fever, chest pain, and pulmonary infiltrates. It may follow bacterial pneumonia. Hypoxemia may develop rapidly, causing dyspnea. 