Extramedullary Hematopoiesis in the Sinonasal Cavity: A Case Report and Review of the Literature

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.

Extra-Medullary Hematopoiesis in Sickle Cell Disease Presenting as a Right Adrenal Mass

“…Common sites of EMH include the reticuloendothelial system such as spleen, lymph node, and mediastinum; however, the process can include virtually any tissue of the body [5]. Cases have been reported of EMH in the bowel, breast, pleura, and brain [6]. In our literature review, we found cases of retroperitoneal EMH after thalassemia and hereditary spherocytosis [7].…”

Rare sites of extramedullary hematopoiesis involving the bilateral orbits, paranasal sinuses and dura in a patient with acute myeloid leukemia

“…Any sinus can theoretically be affected, but the most commonly affected location was the maxillary sinus. There were 9 cases with maxillary involvement, 6 with sphenoid involvement, and 4 with ethmoid involvement [9] .…”

Extramedullary haematopoiesis in patients with transfusion dependent β-thalassaemia (TDT): a systematic review

“…Extraosseous and paraosseous EMH are the two forms of EMH [ 20 ]. Extraosseous haematopoiesis occurs in organs containing multipotent stem cells, such as the spleen and liver, and produces haematopoietic foci that are not associated with bone marrow.…”

“…Splenomegaly or hepatomegaly, which can present as early satiety, bloating, pressure, or stomach discomfort, is common in patients. Para osseous EMH, in contrast to extraosseous EMH, arises surrounding the bone as a result of hyperactive marrow herniation [ 20 ]. It is more evidently seen in Sickle cell anaemia and thalassaemia as the erythroid marrow turnover is faster.…”