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Nancy Chen,1 Chia-Shuen Lin,2 Yung-Hsiang Hsu,3 Wei-Han Huang,4,5 Chin-Te Huang,6 Yuan-Chieh Lee1 1Department of Ophthalmology, Hualien Tzu Chi Hospital, Hualien, Taiwan; 2Department of Dermatology, Hualien Tzu Chi Hospital, Hualien, Taiwan; 3Department of Pathology, Hualien Tzu Chi Hospital, Hualien, Taiwan; 4Department of Clinical Pathology, Hualien Tzu Chi Hospital, Hualien, Taiwan; 5Department of Hematology and Oncology, Hualien Tzu Chi Hospital, Hualien, Taiwan; 6Department of Ophthalmology, Chung Shan Medical University Hospital, Taichung, TaiwanCorrespondence: Yuan-Chieh LeeDepartment of Ophthalmology, Hualien Tzu Chi Hospital, Buddlist Tzu Chi Medical Foundation, 707 Sec. 3, Chung Yung Road, Hualien, 970, TaiwanTel +886 3 8561825 ext 12218Fax +886 3 8577161Email yuanchieh.lee@gmail.comPurpose: To report a case of an orbital myeloid sarcoma concurrent with JAK2 mutation myelofibrosis, which rapidly transformed into acute myeloid leukemia upon aggressive treatment.Results: A 51-year-old woman had progressive swelling of periorbita for one month. Magnetic resonance imaging demonstrated a well-defined, mild enhanced mass indenting the adjacent right lateral rectus muscle and the globe. Biopsy from anterior orbitotomy revealed an orbital myeloid sarcoma. Bone marrow study showed concurrent myelofibrosis. Although the orbital lesion subsided remarkably under aggressive chemotherapy and radiotherapy, the leukemic transformation was noticed in the third month following the initial presentation.Conclusion: This case demonstrated that myeloid sarcoma should be included in the differential diagnosis of orbital diseases, with or without involvement of hematological disorders. Early diagnosis and aggressive treatment as for AML are crucial as the prognosis is usually poor for adult orbital MS.Keywords: myeloid sarcoma, myelofibrosis, acute myeloid leukemia, JAK2 mutation |