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Case 14

History:

10 year old African American presenting with nasal congestion

Findings:

CT of the paranasal sinuses showing complete obliteration of the paranasal sinuses with curvilinear areas of increased density

Diagnosis:

Extramedullary hematopoesis (in a sickle cell patient).

Discussion:

Extramedullary hematopoesis (EH) is the production of  the normal precursors of blood cells in sites other than the bone marrow cavity.  It is typically encountered in cases of chronic anemia as a result of elevated synthesis of renal erythropoietin. Most often, it is microscopic and asymptomatic, but it can sometimes manifest as organomegaly and tumor-like masses. EH is often encountered with sickle cell disease and can affect the liver and spleen and less frequently the lungs, GI and GU tract, paravertebral region and CNS among other organs. CT sinuses typically show complete sinus opacification with curvilinear striations.

References:

  • P.N.A Reed Keamey, Pathology quiz case 2. extramedullaryhematopoeisis of paranaal sinuses, Arch Otolaryngol head and neck surg 128 (January) 2002 78-79.