First Author: E.Ispizua-Mendivil SPAIN
Co Author(s): M. Mendivil Soto I. Bearan Maria P. Isusi Gorbea J. Lopez Duque P. Urigoitia Ugalde
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To report a case of vitritis secondary to a recurrence of mycosis fungoides, in which vitrectomy with appropiate technique and sample handling was key to achieve a certain diagnosis.
Hospital de Basurto, Bilbao, Spain Departments of Ophthalmology, Pathology, Cytodiagnosis, Dermatology, Hematology.
A 67-year-old woman presented with progressive visual loss in the right eye. Best corrected visual acuity (BCVA) was 20/40. Anterior segment evaluation revealed posterior sinechiae, Tyndall 3+ in the right eye, and vitritis with in the right eye, with no remarkable findings in the left eye. Medical history included skin-limited mycosis fungoides diagnosed 9 years ago, with no evidence of bone marrow involvement. The treatment for the skin affection included photochemotherapy (PUVA), bexarotene, and interpheron therapy. BCVA further deteriorated to 20/200 200 in two weeks time. Diagnostic pars plana vitrectomy was performed to obtain a sample of undiluted vitreous fluid from the right eye. Microscopic examination of the vitreous fluid (vitreous cassette) was also carried out. The specimen was immediately delivered in the appropriate media and temperature to cytopathologist and the hematologist, and the results were ready in less than two hours.
Analysis of a vitreous fluid sample demonstrated a monomorphic population of abnormal medium-sized T-cell lymphocytes, with kidney-shaped nuclear contours, typical of mycosis fungoides. Flow cytometry revealed abnormal immunophenmotype of T lymphocytes, which in nearly 80% of the cases lacked CD3 and CD7 expression, with CD4 present, being this consistent with mycosis fungoides diagnosis. The patient had presented evening fever during some days before the vitrectomy. After having the analysis results, we ordered an urgent cerebral CT-scan that showed no evidence of intracranial involvement. The vision improved after a subTenon trimcinolone injection, however general treatment was palliative due to the lack of response to previous chemotherapy and radiotherapy, and was limited to oral prednisone to control fever.The patient finally died in less than two months.
Recurrent mycosis fungoides may present with vitritis as the first manifestation. Vitrectomy is a very valuable procedure for the diagnosis of uveitis masquerade syndromes including ocular lymphoma. The infusion must be off to obtain 1.5 to 2mL of undiluted vitreous specimen, via aspiration provided by a surgical assistant with a 3-mL syringe. Before the surgery, we must talk to the pathologist and cytodiagnosis laboratory of our institution, about the suspected diagnosis and desired testing, in order to achieve an appropriate handling of the sample and a prompt delivery, in order to increase the likelihood of a diagnosis.