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Clinicopathologic Reports, Case Reports, and Small Case Series
May 1, 2005

Orbital Sclerosing Extramedullary Hematopoietic Tumor

Arch Ophthalmol. 2005;123(5):689-691. doi:10.1001/archopht.123.5.689

Extramedullary hematopoiesis can occur in myeloproliferative disorder, including myelofibrosis,1 chronic myeloid leukemia,2 and, rarely, polycythemia vera.3 When fibroblastic proliferation accompanies a solid mass appearance, such lesions have been termed sclerosing extramedullary hematopoietic tumors(SEMHTs).4 We describe, to our knowledge, the first case of myelofibrosis with bilateral multiple SEMHTs in the orbitae.

A 62-year-old woman sought medical treatment in April 2000 because of a slow-growing left lower-lid mass of 1 year's duration. She had no associated pain, visual loss, or diplopia. Examination disclosed a left lower-lid mass measuring about 3 × 2 × 2 cm. The mass was not tender and was mobile. It had a smooth surface and appeared to be arising from the orbita. The patient's best-corrected visual acuity was 20/30 OU. Measurement with a Hertel exophthalmometer showed a 2-mm proptosis in the left eye. The intraocular pressure was normal in both eyes. We found no relevant afferent pupillary defect or optic nerve dysfunction. Extraocular movement showed marked limitation of depression and moderate limitation of abduction in the left eye. Fundoscopic findings in both eyes were unremarkable, and the optic disc sizes were within normal limits. Multiple palpable cervical and submandibular lymph nodes were present. The patient had a medical history of myelofibrosis that had been followed up and treated with systemic hydroxyurea since 1997. Splenectomy had been performed in 1997 for massive splenomegaly. There was no evidence of acute myeloid leukemia.