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Thoracic extramedullary hematopoiesis in sickle-cell disease
R V Gumbs1, E A Higginbotham-Ford, J S Teal
1Department of Radiology, Howard University Hospital, Washington, DC 20060.
AJR. American Journal of Roentgenology
|November 1, 1987
Summary
Radiographic findings of thoracic extramedullary hematopoiesis (EMH) in adults with sickle cell disease include paraspinal masses. These masses are typically slow-growing and asymptomatic, not requiring aggressive intervention.
Area of Science:
- Radiology
- Hematology
- Oncology
Background:
- Extramedullary hematopoiesis (EMH) is the formation of blood cells outside the bone marrow.
- Thoracic EMH can manifest as paraspinal or paracostal masses.
- Sickle cell disease is a known risk factor for extramedullary hematopoiesis.
Purpose of the Study:
- To describe the radiographic features of thoracic extramedullary hematopoiesis in patients with homozygous sickle-cell disease.
- To evaluate the clinical significance and natural history of these masses.
Main Methods:
- Retrospective review of radiographic findings in eight adult patients with homozygous sickle-cell disease.
- Analysis of imaging characteristics, location, size, and growth patterns of paraspinal and paracostal masses.
- Clinical follow-up data analysis.
Main Results:
- Eight adult patients with homozygous sickle-cell disease presented with unilateral or bilateral paraspinal masses, sometimes with associated subpleural/paracostal masses.
- Masses were well-defined, lobulated, and did not erode adjacent bone.
- Follow-up showed initial rapid growth followed by stabilization or slow growth; patients were asymptomatic.
Conclusions:
- Well-defined paraspinal and/or paracostal masses in patients with sickle cell disease should raise suspicion for extramedullary hematopoiesis.
- These masses are typically slow-growing and asymptomatic, generally not warranting aggressive diagnostic or therapeutic measures.