Summary Richter syndrome describes the development of high‐grade non‐Hodgkin lymphoma (NHL) or Hodgkin lymphoma in patients with chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL). Richter transformation occurs in 3·3 to 10·6% of patients with CLL. The large cell lymphoma clone occurs by transformation of the original CLL clone in the majority of patients, and as a separate and independent neoplasm in fewer cases. Richter transformation may be triggered by viral infections, such as Epstein‐Barr virus infection, which are common in immunosuppressed patients. Trisomy 12 and chromosome 11 abnormalities, as well as multiple genetic defects, have been described in patients with Richter syndrome. These abnormalities may cause CLL cells to proliferate and, by facilitating the acquisition of new genetic abnormalities, to transform into Richter syndrome cells. Presenting features typically include a rapid clinical deterioration with fever in the absence of infection, progressive lymph node enlargement, and an elevation in serum lactate dehydrogenase. Extranodal Richter syndrome has also been reported to occur in the central nervous system, eye, gastrointestinal system, nose, skin, face, bone and bronchus. The therapeutic options include cytoreductive therapy consisting of chemotherapy and immunotherapy, followed by allogeneic stem cell transplantation as postremission therapy.

Late presentation is a major problem in the management of patients with primary open-angle glaucoma. The aim of this study was to determine the clinical features and the degree of visual disability of glaucoma patients at initial presentation. This was a prospective study of new patients with a diagnosis of primary open-angle glaucoma seen between January 2001 and August 2002 at the University of Benin Teaching Hospital, Nigeria. The patients were analysed in the context of age, gender, geographical abode, educational and socioeconomic status and clinical presentation. The patients were examined using the Snellen's chart, pen torch, slit lamp biomicroscopy, ophthalmoscopy, tonometry, perimetry and gonioscopy. Over a 20-month period 154 patients were studied; the mean age was 52.73 years (standard deviation +/- 15.98) and the highest incidence was in the seventh decade. The male to female ratio was 2.1:1. In all, 119 patients (77.3%) presented with subjective visual loss in one or both eyes, while 57.1% dated the onset of their symptoms to 1-5 years before presentation. Overall, 38 patients (24.7%) were blind when using visual acuity as the criteria, while 87 patients (56.5%) were blind when using visual fields as the criteria. The problem of late presentation has not improved over the last two and a half decades. More measures towards early detection by extensive health education, screening programmes and training of more eye care providers need to be put in place.

No abstract

Background: Ophthalmic involvement in lymphoma is a relatively rare condition that can result from a primary intraocular lymphoma or an intraocular manifestation of systemic lymphoma. This report reviews the ophthalmic manifestations of lymphoma. Methods: Review of relevant information from journal articles and Internet search. Results: Almost all the structures in the orbit, adnexiae and eye can be involved in lymphoma. Lymphoma of the eye and adnexiae are most frequently of B lineage. Most of the ocular manifestations frequently masquerade as other more benign intraocular conditions including allergic or infectious conjunctivitis, uveitis, multiple evanescent white dot syndrome, acute retinal necrosis or herpetic retinitis. Correct diagnosis thus depends on a high index of suspicion and frequently requires radiologic imaging, histologic analysis, particularly vitreous biopsy or flow cytometry, subretinal aspiration and retinal biopsy. Diagnosis is often delayed and may lead to a fatal outcome. Recognition of its modes of presentation facilitates early diagnosis and treatment that may improve prognosis. Conclusions: It is important to review the ocular manifestations of lymphoma to assist the ophthalmologist to play a pivotal role in the prompt diagnosis and treatment of ocular lymphoma, and the haematologist/oncologist to recognize the need for a complete ophthalmic evaluation in the diagnosis, follow-up and management of lymphoma patients.Key words: lymphoma, ophthalmic manifestations, masquerade, vitreous biopsy Résumé Introduction: La participation ophtalmique dans lymphome est une condition rélativement rare qui peut être provoqué par lymphome intraoculaire primaire ou une manifestation intraoculaire du lymphome systématique. Ce rapport fait une rétrospective des manifestation ophtalmique du lymphome. Méthodes: Une rétrospective des informations pertinentes des articles dans les journaux et des recherches dans l'internet. Résultats: Prèsque toutes les structures dans l'orbite, adnexiae et l'oeil peuvent participer dans lymphome. Lymphome de l'oeil et d'adnexiae sont le plus souvent du lignage B. La plupart des manifestations oculaires les plus souvent mascarade comme d'autre conditions intraoculaire plus bénigne y compris des infections conjunctivite ou allergique, uveite, syndrome tacheté du blanc évanescent multiple, nécrose aigue rétine ou rétinite herpetique. Donc diagnostique correcte dépend d'index plus élevé du soupçon et l'imaging radiologique est très fréquemment éxigé, analyse histologique, la biopsie vitreouse en particulier ou bien écoulement cytométrie, aspiration sousrétine et la biopsie rétine. Le diagnostique est souvent rétardé et pourrait aboutir à un résultat sérieux. Réconnaissance de son mode de présentation facilitera un diagnostique précoce et un traitement qui pourrait améliorer la prognose. Conclusion: C'est très important de faire une rétrospective des manifestations oculaires du lymphome afin d'aider l'ophtamologiste de jouer un rôle clef dans un diagnostique précoce et t...