Splenic autoinfarction: Absent or small calcified spleen
• Extramedullary hematopoiesis: Most commonly paravertebral soft tissue masses of homogeneous density
• Findings of iron deposition due to repetitive transfusions








IMAGING
General Features
CT Findings
• Spleen
• Extramedullary hematopoiesis: May have many different manifestations, including paravertebral soft tissue masses (homogeneous soft tissue density), hepatosplenomegaly, or perinephric soft tissue “rind” encasing kidneys
MR Findings
• Persistence of red (cellular) marrow in bones with low-signal marrow on T1WI
Demand for increased production of RBCs (due to RBC destruction and anemia) prevents normal conversion of red to yellow marrow

PATHOLOGY
General Features
• Genetics
Sickle trait results when patient has only 1 abnormal sickle cell gene (30-40% of cases)
Some patients may have 1 copy of sickle cell hemoglobin gene and 1 copy of another abnormal hemoglobin molecule (such as β-thalassemia)


CLINICAL ISSUES
Presentation
• Most common signs/symptoms
Acute, painful vasoocclusive crisis (may occur several times each year)




























