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مقاله
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Abstract
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Title:
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Ophthalmic Manifestations of Rosai-Dorfman Disease in 5 Patients
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Author(s):
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Babak Masoomian,Sara E. Lally, Jerry A. Shields, Carol L. Shields
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Presentation Type:
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Oral
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Subject:
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Ophthalmic Plastic and Reconstructive Surgery
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Others:
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Presenting Author:
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Name:
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Babak Masoomian
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Affiliation :(optional)
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Wills Eye Hospital, Philadelphia, USA
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E mail:
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dr.masoomian@yahoo.com
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Phone:
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44654668
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Mobile:
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09059236165
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Purpose:
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To report clinical features, pathology characteristics and treatment outcomes of five patients with Rosai-Dorfman disease (RRD).
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Methods:
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A retrospective case series of all cases with RRD seen in oncology service of Wills Eye Hospital from 1974 to 2018 (n=5).
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Results:
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There were five patients (3 male and 2 female) and 6 eyes with ophthalmic manifestations of RRD. The mean age at initial presentation was 32.8 (range 10-52) years. All patients had unilateral disease except one. The mean duration of symptoms was 9 (range 5-24) months. The ocular tissue affected by RRD included the conjunctiva (n=4), cornea (n=2), orbit (n=2) and uvea (n=2). Palpable lymphadenopathy was detectable in 2 patients . Patient with bilateral disease and uveitis had diffuse lymphadenopathy and multiorgan involvement. Patients with orbital masses had no systemic involvement (n=1) or lymphadenopathy with paranasal sinusitis (n=1). None of the patients with perilimbal lesions had lymphadenopathy (n=2) but, paranasal sinusitis was detected in one of them. Of six eyes with a mean follow-up of 31 months (range, 10-76 months) after surgery, tumor regression was achieved in all cases without recurrence.
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Conclusion:
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Rosai-Dorfman Disease can occur in and around the ocular tissues. Patients with intra-ocular manifestations and/or bilateral disease usually have multiorgan systemic involvements while, most patients with conjunctival or orbital lesions have limited disease
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Attachment:
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