Ann Hematol (2001) 80:311–313 DOI 10.1007/s002770000263
C A S E R E P O RT
M. Reiser · A. Josting · V. Diehl · A. Engert
Primary lymphoma of the conjunctiva – a rare manifestation of indolent non-Hodgkin’s lymphoma
Received: 12 January 2000 / Accepted: 11 October 2000 / Published online: 20 February 2001 © Springer-Verlag 2001
Abstract Lymphoid tumours of the conjunctiva are extremely rare. Such tumours can appear as an isolated neoplasm or as part of a systemic disease. We describe the clinical and histopathologic findings of a patient with bilateral conjunctival lymphoma who presented with a 5-year history of chronic conjunctivitis. After clinical staging, including (1) thoracic and abdominal computerised tomography scans, (2) magnetic resonance tomography of the brain and orbita, (3) fibre-optic gastrointestinoscopy, (4) colonoscopy and (5) bone marrow biopsy, no other foci of the lymphoma were found. The patient received radiation therapy and achieved complete remission. We conclude that conjunctival lymphoma should be included in the differential diagnoses of chronic conjunctivitis. Persisting signs and symptoms of conjunctivitis not responding to standard treatment should prompt biopsy. Keywords Non-Hodgkin’s lymphoma · Lymphoma · Conjunctiva · Treatment
Introduction Primary non-Hodgkin’s lymphoma of the conjunctiva are extremely rare and only a few cases are reported in the literature [1, 2, 9, 10, 14, 16]. In addition to these case reports, only two studies with larger number of patients, involving a total number of 50 cases could be identified [5, 8]. Although primary ocular lymphomas may be found in the conjunctiva, eyelids and lacrimal glands, the majority occurs in the orbit. Most of the patients with conjunctival lymphoma present with a localised unilateral stage I,E or bilateral stage II,E. Radiotherapy is usually considered the treatment of choice in such situaM. Reiser (✉) · A. Josting · V. Diehl · A. Engert First Department of Internal Medicine, Cologne University Hospital, Joseph-Stelzmann-Str. 9, 50924 Cologne, Germany e-mail:
[email protected] Tel.: +49-221-4786628, Fax: +49-221-4783531
tions. The clinical course is indolent with most of the patients failing to develop extra-ocular lymphoma. Patients may present with slowly evolving symptoms resembling inflammation refractory to standard treatment. A biopsy has to be taken in these cases. The diagnosis is often difficult requiring immunohistology to distinguish malignant from benign lymphoid infiltrates.
Case report A 55-year-old woman was referred to our department with symptoms of conjuncitivitis of both eyes. She presented with an indolent red-coloured tumour of the epibulbar conjunctiva of her left eye and indolent redness of the right eye. Similar changes had appeared in her left eye, 5 years previously, with the tumour starting to develop 2 years later. The condition in her right eye had developed slowly over a period of 2 years with a gradual swelling of the epibulbar conjunctiva. A biopsy of the tumour was taken in the Department of Ophthalmology and diagnosed by the local pathologist as a nonmalignant lymphoid infiltration of the conjunctiva. Because of the patently aggressive growth pattern, a second examination by a lymphoma reference pathologist was deemed necessary and revealed a malignant infiltration of a low-grade non-Hodgkin’s lymphoma. Histological examination showed a diffuse infiltration of the conjunctiva with atypical mostly small lymphocytic cells with cleaved nuclei and large cells in addition to a few morphologically normal plasma cells. Atypical cells stained positively for CD20, CD10 and Ki-B3 and showed strong expression of Bcl 2. Morphologically normal plasma cells stained positively for polyclonal kappa and lambda light chains; centroblastic and centrocytic lymphoma cells did not show any expression of light chains. The subtype of the lymphoma was classified as centroblastic-centrocytic according to the up-dated Kiel classification and as follicle centre cell lymphoma grade II (
