Extramedullary Hematopoiesis in the Sinonasal Cavity: A Case Report and Review of the Literature. (April 2020)

• Record Type: Journal Article
• Authors: Clark, Carly A.; Worden, Cameron P.; Thorp, Brian D.; Ebert, Charles S.; Zanation, Adam M.; Senior, Brent A.; M. Johnson, Steven; McClain, Wade G.; Kimple, Adam J.
• Is Part Of: Allergy & rhinology. Volume 11(2020)
• Publication Date: 2020-04
• Subjects: extramedullary hematopoiesis -- sickle cell anemia -- paranasal sinus -- sinonasal mass -- sinus tumor; Allergy -- Periodicals; Otolaryngology -- Periodicals; Nose -- Diseases -- Periodicals; Allergy; Nose -- Diseases; Otolaryngology; Hypersensitivity -- Periodicals; Otolaryngology -- Periodicals; Nose Diseases -- Periodicals; Periodicals; Electronic journals; 616.97005
• DOI: 10.1177/2152656720918874
• Languages: English
• ISSNs: 2152-6575

Abstract

Background: Extramedullary hematopoiesis (EMH) occurs in patients with hematologic disorders, but rarely within the paranasal sinuses. We report a case of EMH in a 17-year-old male with sickle cell disease (SCD) who presented with occipital pain and sinusitis. A computed tomography (CT) scan demonstrated heterogeneous opacification of the right maxillary sinus concerning for allergic fungal sinusitis or a fungal ball with bony erosion. He was taken to the operating room for endoscopic biopsy and a limited endoscopic sinus surgery. Grossly, his maxillary sinus was filled with spiculated osseous tissue. Final pathology demonstrated active hematopoietic bone marrow filling the sinus. Methods: We present a case report and literature review of sinonasal EMH. Results: We identified 14 articles with 15 patients. EMH was typically associated with SCD or beta thalassemia. The average age of presentation was 30. There was a male sex predilection with a ratio of 11:15. The most common presenting symptom was a headache and nasal obstruction (33% for both). The most common finding on CT was a soft tissue expansile mass (73%). The most commonly affected location was the maxillary sinus (60%). Conclusions: This case report serves as a reminder to consider EMH as an uncommon cause of sinus opacification, particularly in patients with SCD or beta thalassemia. The expansion of hematopoietic tissue may be identified as a sinus mass on CT. By recognizing the potential manifestations of chronic anemia, an accurate and timely diagnosis can be made.