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.

No abstract

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.

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...

*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.

The clinicopathologic features of malignant lymphomas had not been documented in the Niger Delta region of Nigeria, which is known for its petrochemical industries and gas flare sites. Cases of lymphomas that presented to the University of Benin Teaching Hospital (UBTH), a major referral centre in the region, from January 1990 to December 2003 were reviewed. Demographic and clinical information were obtained from the case files. Diagnosis was established based on the histological examination of an accessible biopsy lymph node and classified according to the Working Formulation (WF). Haematological parameters were done using an automated Coulter counter. Non-Hodgkin's lymphoma (NHL) occurred predominantly in young adults (20-39 years). A majority of the patients presented in the advanced stage of the disease (Stages III-IV) according to the Ann Arbor system and a performance status (PS) scale of 2-4. The intermediate grade NHL (41.2%) formed the largest group of which diffuse large cell lymphoma (DLCL) was the most commonly observed histopathologic type followed by the large cell immunoblastic type.

Context:Leukemias may present with, or be associated with ocular disorders.Aims:To determine the rates of ophthalmic disorders in adult patients with leukemia.Settings and Design:A prospective study of ocular disorders in adult patients with leukemia at the University of Benin Teaching Hospital, Benin City, Nigeria, between July 2004 and June 2008 was conducted.Methods and Materials:The patients were interviewed and examined by the authors and the ocular findings were recorded. Statistical analysis was performed using Instat GraphPad™ v2.05a statistical package software. The means, standard deviation, and the Kruskal-Wallis non parametric test were performed.Results:Forty-seven patients with leukemias were seen. Nineteen patients (40.4%) had CLL, 14(29.8%) had CML, 9(19.1%) had AML and 5(10.6%) had ALL. Seven patients (14.9%) had ocular disorders due to leukemia. The ocular disorders due to the leukemia were proptosis in two patients (4.3%), retinopathy in one patient (2.1%), conjunctival infiltration in one patient (2.1%), periorbital edema in one patient (2.1%), retinal detachment in one patient (2.1%), and subconjunctival hemorrhage in one patient (2.1%). There was no significant difference in rate of the ocular disorders in the various types of leukemia (Kruskal-Wallis KW= 4.019; corrected for ties. P=0.2595). One patient (2.1%) was blind from bilateral exudative retinal detachment while 1 patient (2.1%) had monocular blindness from mature cataract.Conclusions:Ophthalmic disorders that are potentially blinding occur in leukemias. Ophthalmic evaluation is needed in these patients for early identification and treatment of blinding conditions.

Haematological values were determined in 246 sickle cell anaemia (SCA) patients in three centres in Benin City, Nigeria, as well as 84 control subjects with haemoglobin A (HbAA) confirmed by haemoglobin electrophoresis at pH 8.6. Automated Coulter counter was used to determine the complete blood counts while the foetal haemoglobin was estimated by the modified Betke method, and haemoglobin A 2 by the HbS-free microcolumn chromatography. Six patients (2.4%), comprising of two males and four females, out of the 246 SCA patients were found to have elevated haemoglobin A 2 (>3.5%). All the six patients also had elevated haemoglobin F (>1.5%). The family members of three of these six patients were successfully screened. These three patients (1.2%) were found to have positive co-inheritance of thalassaemia trait and sickle cell anaemia. The erythrocyte indices were all reduced in these selected families except for one family whose mean cell haemoglobin concentration was within normal range. Peripheral blood film revealed the presence of target cells and occasional microcytes apart from the sickled cells. The possibility of co-inheritance of the beta thalassaemia gene with the sickle cell gene occurs in about 1.2% of Nigerians with sickle cell anaemia.