Volume 4, Issue 1 , Pages 32-35, January 2009
Extramedullary hematopoiesis in the maxillary sinus
Article Outline
Summary
Chronic hemolytic anemia may result to compensatory extramedullary hematopoiesis. We present the diagnostic and treatment challenges of an unusual case of extramedullary hematopoiesis involving the maxillary sinus. Review of literature is performed.
Keywords: Extramedullary hematopoiesis, Paranasal sinuses
1. Introduction
Sickle cell disease is one of the most common hemoglobinopathy worldwide. It results from an amino acid substitution in the beta chain of hemoglobin. It affects mostly African Americans and individuals of Mediterranean decent. In those with homozygous sickle cell trait, the chronic hemolytic anemia results in a compensatory extramedullary hematopoiesis. We present the diagnostic and treatment challenges of a rare case of extramedullary hematopoiesis (EH) involving the maxillary sinus.
2. Case report
A 12-year-old African American male with sickle cell disease (SCD) presented to our ear, nose and throat clinic complaining of nasal congestion and snoring for 1 year. The patient had been treated with various nasal sprays without improvement. Physical examination including nasal endoscopy revealed obstructive adenoid hyperplasia. A non-contrast axial and coronal CT scan of the paranasal sinuses confirmed obstructive adenoid hyperplasia. In addition, there was complete opacification of the left maxillary sinus with some bulging of the medial wall and protusion of soft tissue into the left osteo-meatal complex region (Fig. 1, Fig. 2). Pre-operative radiographic differential diagnosis included antro-choanal polyp, sinusitis or mucous retention cyst involving the left maxillary sinus.
Fig. 1.
Axial view of CT scan of paranasal sinuses.
Fig. 2.
Coronal view of CT scan of paranasal sinuses.
On presentation, the patient had hemoglobin 8.9
g/dL, hematocrit 25.5%, white cell count 23,000×109L−1 and platelet count of 703,000×109L−1. The patient was admitted and underwent intravenous hydration and blood transfusion in preparation for surgery. The next day the patient underwent adenoidectomy uneventfully followed by left Caldwell approach to the left maxillary sinus. During removal of the anterior wall of the maxillary sinus, significant bleeding occurred originating from a maxillary mass. After hemostasis was achieved using surgical packing, the sinus cavity was examined and revealed a soft mass. Preliminary intra-operative frozen section incorrectly identified the mass as fibrous dysplasia. However, final pathology of the specimen demonstrated EH tissue (Fig. 3). The patient's post-operative recovery was uneventful. No further treatment was recommended. At his follow-up visit the patient reported improved nasal breathing.
Fig. 3.
Erythroids (magnification 40×).
3. Discussion
Extramedullary hematopoiesis is the production of the normal precursors of blood cells in sites other than the long bones, pelvis, spine and sternum. It occurs in chronic hemolytic anemias as a response to increased renal production of erythropoietin consequent to anemia. EH is a compensatory mechanism that results when stressed bone marrow is unable to keep up with the body's requirements because of increased demand or ineffective erythropoiesis [1]. EH is a common manifestation of SCD and primarily occurs in the liver and the spleen where fetal hemoglobulin is normally produced. It less frequently occurs in other organs such as the lungs, gastrointestinal and urinary tract, adrenal glands, peritoneum, skin, breast, central nervous system and paravertebral areas [2], [3]. Initially, EH is microscopic and clinically silent but may stimulate the production of enough cells to cause organomegaly or tumor-like masses with symptoms associated with the organ or system involved.
Two types of EH have been described in the literature: (1) paraosseous foci and (2) extraosseous foci [4]. Patients with paraosseous foci have herniation of medullary tissue from underlying bone. This is usually seen in patients with SCD and thalassemia where marrow activity is high. Patients with extraosseous EH have soft tissue hematopoetic foci that arise from multipotent stem cells. This occurs in patients with myelofibrosis of tumor marrow destruction where marrow activity is ineffective [5]. Paraosseous EH is the likely mechanism of the paranasal sinuses, and is the source of the soft tissue noted within the paranasal sinuses. A markedly expanded diploetic space herniates into the sinus cavity and gives the radiologic appearance of the sinuses being filled with a soft tissue mass [6].
EH rarely involves the facial bones in the homozygous sickle cell patient but when present, the mandible and orbital roof are the most common sites [6]. When the maxillary sinus is involved, expansion of the marrow space of the maxillary sinus wall into the sinus cavity space may result in hematopoetic cells within the sinus cavity. EH may occur more frequently in sickle cell patients than is reported and may be misdiagnosed as sinusitis.
Four cases of paranasal sinus EH have been reported previously (Table 1). All patients are male with age ranging from 28 months to 17 years. Symptoms at presentation are variable and depend on the location of the extramedullary hematopoiesis. In the majority of cases the initial diagnosis was incorrect; the correct diagnosis required biopsy. EH was first described by Andreou et al. [7] in a 10-year-old male patient with beta thalassemia who presented with seizures and progressive aphasia. Paranasal sinus CT showed expansile masses in the maxillary and ethmoid sinuses bilaterally. Fernandez et al. [6] reported a 28-month-old male with sickle cell disease who had been treated for sinusitis without success. Biopsy showed hyperplastic bone marrow. Joseph et al. [8] describe the case of a 18-year-old boy with beta thalassemia and sickle cell disease who presented with a 4-month history of left focal motor seizures. The CT scan showed complete opacification of the sphenoid sinus. Transnasal biopsy confirmed the final diagnosis. Collins [9] described the case of a 13-year-old African American male with SCD who presented with facial pain and nasal congestion. He underwent endoscopic sinus surgery with the presumptive clinical and radiological diagnosis of allergic fungal sinusitis. Surgery was aborted due to the results of frozen section, which revealed erythroid and myeloid precursors.
Table 1.
| Study | Patient's characteristics | Symptoms | Radiology findings | Initial misdiagnosis | Intervention |
|---|---|---|---|---|---|
| Index | 12 years old, male, SCD | Nasal obstruction | Right maxillary sinus mass obstructing the right osteomeatal complex | Polyp, sinusitis, retention cyst. Frozen section: fibrous dysplasia | Right endoscopic sinus surgery |
| Andreou et al. [7] | 10 years old, male, beta-thalassemia | Seizures, progressive aphasia | Expansile masses bilateral maxillary and ethmoid sinuses | No data | No data |
| Collins et al. [9] | 13 years old male, SCD | Nasal obstruction, bifrontal headache, rhinorrhea, facial pain | Compete opacification of maxillary and anterior ethmoids sinuses with intrasinus calcifications. No bony erosion | Fungal sinusitis | Endoscopic sinus surgery |
| Fernandez et al. [6] | 28 months old male, SCD | Rhinorrhea | Maxillary sinus opacification and enlargement | Sinusitis, infarction | Left maxillary sinus biopsy |
| Joseph et al. [8] | 18 years old male, beta-thalassemia, SCD | Left focal motor seizures | Dense lesion in right half of sphenoid sinus | Transnasal biopsy |
Though CT findings may be suggestive of EH in patients with chronic anemia, definitive diagnosis requires biopsy. Nonspecific findings of sinus opacification and sinus wall expansion may be noted. Facial deformities may result in some cases [1].
Treatment of EH involving the sinus cavity is not recommended as this may be a vital part of the patient's functioning hematopoietic tissue [9]. Extramedullary hematopoiesis in other regions such as the spine is treated with surgery with or without radiation when patients have compression symptoms.
4. Conclusions
Extramedullary hematopoiesis should be considered in the differential diagnosis of any paranasal sinus mass in patients with chronic anemia. Otolaryngologists, radiologists, and pathologists should be aware of this entity in order to improve diagnostic and management approaches to these patients.
References
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- . Extensive extramedullary hematopoiesis in a thyroid nodule. Mod Pathol. 1996;9:1062–1065
- . Extramedullary hematopoiesis of the middle ear in a patient with thalassemia. Am J Otolaryngol. 1998;19:287–289
- . Tc-99m nanocolloid scintigraphic imaging of intracranial meningeal extramedullary hematopoiesis in a patient with idiopathic myelofibrosis. Ann Nucl Med. 2006;20:565–568
- . Computed tomography of intracranial extramedullary hematopoiesis. J Comput Assist Tomogr. 1984;8(August (4)):788–790
- . Maxillary sinus marrow hyperplasia in sickle cell anemia. Pediatr Radiol. 1995;25(Suppl. 1):S209–S211
- . Bone marrow hyperplasia of the maxillary sinuses in beta-thalassemia. J Comput Assist Tomogr. 1984;8:180
- . Haematopoietic tissue presenting as a sphenoid sinus mass: case report. Neuroradiology. 2000;42:153–154
- . Extramedullary hematopoiesis of the paranasal sinuses in sickle cell disease. Otolaryngol Head Neck Surg. 2005;132:954–956
PII: S1871-4048(08)00041-5
doi:10.1016/j.pedex.2008.05.007
© 2008 Elsevier Ireland Ltd. All rights reserved.
Volume 4, Issue 1 , Pages 32-35, January 2009
