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.
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...
Sixty patients were prospectively studied with the aim of analyzing the clinical and laboratory features and outcome of patients diagnosed with chronic lymphocytic leukaemia (CLL) in a major referral center in Niger Delta region of Nigeria for 10 years (1995-2005). The peripheral blood, bone marrow cytology, clinical features and stage at diagnosis were studied. Treatment modalities, response to treatment and survival outcome of the patients were analysed. Sixty patients (15 men and 45 women) were seen, with female preponderance (M : F ratio,1 : 3). The CLL incidence was 36.4% of total leukaemias. The median age was 56 years with peak age group at 51-60 years while 15% were below 40 years. Major clinical findings include lymphadenopathy (91.7%), anaemia (58.3%), abdominal swelling (58.3%), and splenomegaly (50%) with 53 patients (88.3%) presenting as International (Binet) stage B and C while only seven patients (11.7%) were seen in stage A. The least clinical presentation includes Richter's syndrome in 3.3% of cases and herpes Zoster skin manifestations in two patients (3.3%). There was a strong association between the blood counts at diagnosis and outcome of therapy. The 2-year survival for young (<55 years) and older (>55 years) CLL patients was 27.2% and 28.9%, respectively, which is still very poor because of a number of strong limiting factors. CLL is not rare in Southern Nigeria and its presentations are similar to cases seen worldwide. Contrary to existing literature a female predominance was observed in this study with majority of patients seeking medical intervention late. It is therefore recommended that future research into the genetic make up/HLA typing of patients of African descent is needed to clarify some of the differences observed.
*The increased use of chemotherapeutic agents has resulted in longer cancer patient survival. Consequently the ophthalmologist is seeing more patients with adverse ocular side effects secondary to these antineoplastic agents. Ocular toxicity induced by cancer chemotherapy includes a broad spectrum of disorders, reflecting the unique anatomical, physiological and biochemical features of the eye. Understanding the ocular side effects will assist the ophthalmologist and oncologist to recognize them early and intervene before blindness occurs. Anticipation of various treatmentrelated toxicities may also provide the opportunity for pharmacists to develop intervention strategies that could minimize or eliminate an expected side effect. The ophthalmologist should examine patients on anticancer therapy at baseline and three monthly thereafter. The various ocular side effects of anticancer chemotherapeutic agents, tamoxifen, and interferon on the adnexia, anterior segment, posterior segment and neuro-ophthalmic structures were reviewed.
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