Extramedullary hematopoiesis occurring as a nasal polyp in a man with a myeloproliferative disorder.

Abstract

We describe the case of a patient with a known myeloproliferative disorder who presented with epistaxis and what clinically appeared to be a nasal polyp. The mass was resected and proved to represent a focus of extramedullary hematopoiesis. The patient subsequently developed extramedullary hematopoiesis of the skin and the stomach wall. Following nasal polypectomy, he did well for a time, but he eventually died as a result of other complications of his disease. This unique case serves as a reminder that common rhinologic complaints can be a sign of significant and life-threatening pathology.

Introduction

Nasal polyps are a common finding in patients who present with both epistaxis and nasal obstruction. Polyps that fail to respond to medical therapy are best treated by surgical excision, which is generally considered to be the definitive treatment. However, when polyps are characterized by atypical features, evaluation for systemic disease is warranted.

Extramedullary hematopoiesis has been reported in patients with myeloproliferative disease. In fact, splenomegaly seen with this disorder is generally the result of extramedullary hematopoiesis in the spleen. In this article, we report an unusual case of extramedullary hematopoiesis in a nasal polyp that resulted in significant and potentially life-threatening epistaxis. The polyp was removed under platelet coverage. However, had we known beforehand that the polyp was caused by extramedullary hematopoiesis, we might have managed it more safely with radiation therapy.

Case report

A 72-year-old white man first presented in September 1992 with a suspected myeloproliferative disorder. His white blood cell count (WBC) was elevated (10,400/ [mm.sup.3]) and he had monocytosis. Analysis of serial bone marrow specimens ultimately revealed the presence of fibrosis in the bone marrow as well as dysplastic features, indicating that the patient had a hybrid myeloproliferative and myelodysplastic syndrome, probably chronic myelomonocytic leukemia. Findings on his initial head and neck examination were normal; his mucous membranes were clear and he exhibited no sign of nasal polyps. Moreover, his chest was clear, he exhibited no adenopathy, there was no detectable hepatosplenomegaly on abdominal examination, and his neurologic examination was negative. He was managed with supportive therapy. In January 1996, he was diagnosed with prostate cancer, and he underwent a radical prostatectomy.

By 1997, the patient's WBC had risen to 17,600/[mm.sup.3], and by October 1998, it was 43,000/[mm.sup.3]. In January 1999, he required treatment with hydroxyurea when his WBC exceeded 50,000/[mm.sup.3]. In the meantime, he also gradually developed splenomegaly, and in November 2000 he underwent a splenectomy to relieve left upper-quadrant discomfort. He also experienced progressive thrombocytopenia (his platelet count fell to as low as 30,000/[mm.sup.3]); he was treated with a course of amifostine in November 2001, but he did not respond. He was also treated with interferon alfa in an attempt to control his hyperleukocytosis, but again he did not experience a good response, as his WBC approached 100,000/[mm.sup.3].

In December 2001, the patient developed difficulty breathing through his nose. Endoscopy revealed that a right nasal polyp had arisen from the superior meatus. He was initially treated with a steroid nasal spray but did not respond. Findings on computed tomography of the sinus were unremarkable. The polyp was removed under platelet coverage (his platelet count was down to 20,000/[mm.sup.3]) in January 2002.

The gross pathology specimen exhibited features quite typical of an inflammatory nasal polyp, but the microscopic features were most unusual. There was a prominent component of large atypical cells within the dilated vascular channels (figure, A), which initially raised the possibility of an intravascular lymphoma or sarcoma. The cells stained with CD31 (figure, B) and glycophorin, which indicated the presence of a mixture of megakaryocytic and erythroid cells. These findings suggested a diagnosis of extramedullary hematopoiesis.

[FIGURES A-B OMITTED]

The patient subsequently developed nausea and vomiting in addition to a skin rash. Biopsy of the rash detected extramedullary hematopoiesis in the dermis. Biopsy of the gastric wall performed during endoscopy also revealed evidence of extramedullary hematopoiesis. Thereafter, the patient's course went steadily downhill. He was treated with thalidomide and synthetic erythropoietin, but he died of complications of his disease in September 2002.

Discussion

To our knowledge, ours is the first reported case of a nasal polyp containing extramedullary hematopoiesis in a patient with an underlying myeloproliferative disorder.

Although nasal polyps are generally benign and innocuous, they can represent a more serious problem, such as inverting papilloma, adenocarcinoma, and other neoplastic diseases. Being alert to the potential development of such problems in patients with underlying myeloproliferative diseases such as chronic myelogenous leukemia or myelofibrosis might prevent unnecessary surgery. This is especially true in a patient such as ours, in whom there was subsequent evidence of extramedullary hematopoiesis at other sites. Radiation is a very effective treatment for extramedullary hematopoiesis, and early recognition of the possibility that such loci of tissue can arise in the upper aerodigestive tract might render unnecessary a surgical approach and its attendant risk of bleeding. Radiation would have been quite effective as a treatment for our patient's lesion.

Extramedullary hematopoiesis is a well-known complication of myeloproliferative diseases. (1) Because these diseases lead to inefficient hematopoiesis, we often see compensatory splenomegaly or hepatomegaly as a result of loci of hematopoiesis in these organs. Foci of hematopoiesis have also been reported to occur in midline structures such as the mediastinum and retropentoneum. Our patient--who experienced cutaneous, gastric, and nasal mucosal involvement--had a long-standing myeloproliferative disorder that over time resulted in the development of compensatory foci of marrow in multiple organs.

The therapeutic options in such a setting are either to administer systemic chemotherapy or to consider radiation therapy. The former was not possible in our patient because of his attendant thrombocytopenia. Radiation, however, would have been an excellent palliative treatment because it yields nearly a 100% response rate in this situation.

Extramedullary hematopoiesis of the head and neck is rare. The literature contains reports of only six previous cases--three involved the middle ear, (2-4) one involved the thyroid, (5) one involved the esophagus, (6) and one involved the paranasal sinuses. (7) Our case represents the first manifestation of extramedullary hematopoiesis in the nasopharynx. As modern supportive care continues to prolong survival in such patients, clinicians can expect to see atypical presentations such as this one. A high index of clinical suspicion is necessary to detect these rare cases.

References

(1.) da Silva MA, Moriarty A, Schultz S, Tricot G. Extramedullary disease in myelodysplastic syndromes. Am J Med 1988;85: 589-90.

(2.) Giltman LI, Kavanagh KT, Babin RW, et al. Extramedullary hematopoiesis within the hypotympanicum presenting as a glomus tympanicum. Am J Otol 1986;7:218-20.

(3.) Applebaum EL, Frankel A. Extramedullary hematopoiesis of the middle ear. Am J Otolaryngol 1989;10:287-90.

(4.) Meara JG, Potter C, Goodman M, Vernick D. Extramedullary hematopoiesis of the middle ear in a patient with thalassemia. Am J Otolaryngol 1998;19:287-9.

(5.) Lazzi S, Als C, Mazzucchelli L, et al. Extensive extramedullary hematopoiesis in a thyroid nodule. Mod Pathol 1996;9:1062-5.

(6.) Fedeli G, Certo M, Cannizzaro O, et al. Extramedullary hematopoiesis involving the esophagus in myelofibrosis. Am J Gastroenterol 1990;85:1512-14.

(7.) Vargas H, Jennings TA, Galati LT. Unusual paranasal sinus tumors in two patients with common nasal complaints. Ear Nose Throat J 2001;80:724-6, 728-9.

From the Department of Hematology/Oncology, St. Luke Hospital, Fort Thomas, Ky.

Reprint requests: Lawrence V. Brennan, MD, Department of Hematology/Oncology, St. Luke Hospital, 85 Grand Ave., Fort Thomas, KY 41075. Phone: [phone]; fax: [phone]: e-mail: [e-mail]