Amani Al-Kofide

Hodgkin lymphoma in very young children: Clinical characteristics and outcome of treatment

In developed nations, Hodgkin lymphoma (HL) is rare in <5-year olds and represent a minority in developing countries. Little is reported about the biology and behavior of these very young patients compared with older children. 18.75% of our pediatric HL patients (0 – 14 years) were <5 years at diagnosis. This group had more boys, similar incidence of B-symptoms and stage distribution, less mediastinal involvement and bulky disease, and more mixed cellularity subtype than older children. Treatment included chemotherapy (CT; n = 55), combined modality therapy (CMT; n = 12) and XRT only (n = 2). Ten-year EFS and OS was 81.5% and 90.4%, respectively, versus 75.5% and 90.5% for older children (p > 0.5). A trend toward better survival was seen with CMT, using very LD-XRT, than with CT (OS 100% vs. 86.4%[p = 0.3]; EFS 90.9% vs. 81.0%[p = 0.4]). Although CT could be effective in a subset of LR patients, LD-XRT may be needed to effectively treat most of these patients. This dose reduction may reduce XRT-related toxicity, which can be significant in very young children.

High throughput tissue microarray analysis of FHIT expression in diffuse large cell B-cell lymphoma from Saudi Arabia.

Recent studies have suggested a potential prognostic role of alterations of the fragile histidine triad (FHIT) gene in diffuse large B-cell lymphoma. To evaluate possible mechanisms of FHIT inactivation and to further clarify its potential prognostic relevance, we analyzed a set of 114 diffuse large B-cell lymphoma with clinical follow-up information. Tissue microarrays were analyzed by immunohistochemistry for protein expression, and corresponding DNA samples were analyzed for FHIT promotor hypermethlyation. Reduced or absent FHIT expression was found in 75 of 114 diffuse large B-cell lymphoma (66%), but was unrelated to clinical tumor stage or patient prognosis. FHIT promotor hypermethylation was observed in 29 of 93 (23%) interpretable diffuse large B-cell lymphoma. Hypermethylation was not significantly correlated to protein expression loss, which could be explained by competing mechanisms for FHIT inactivation in a substantial fraction of non FHIT hypermethylated diffuse large B-cell lymphoma. Hypermethylation was significantly associated with poor prognosis of diffuse large B-cell lymphoma patients and predominantly seen in nongerminal center diffuse large B-cell lymphoma (27%), but less frequent (13%) in germinal center diffuse large B-cell lymphoma. In summary, these data suggest that promotor hypermethylation is responsible for reduced FHIT expression in a substantial subset of diffuse large B-cell lymphoma, which is primarily composed of nongerminal center subtype with poor patient prognosis.

Yolk sac tumor: histopathologic report of 2 cases

CAN J OPHTHALMOL—VOL. 43, NO. 1, 2008 125 culation time was 18 seconds, and the retinal arteriovenous transit time was 6 seconds. One month later, the patient’s visual acuity was still no light perception, and he had not recovered from the macular necrosis. A case of macular toxicity due to inadvertent intravitreous injection of a high dose (40 mg/0.1 mL) of ganciclovir has been reported.1 However, our case involved a macular infarction following intravitreal ganciclovir treatment at the frequently used dose of 2 mg/0.05 mL in a patient with ARN. Central retinal vein occlusion2,3 is known to be caused by ARN, but, in our case, the injured area was confined to the macula, and the fluorescein angiography revealed no increase in the arm-to-retina circulation time or the retinal arteriovenous transit time. This suggests that central retinal vein occlusion or central retinal artery occlusion is unlikely to be the cause of the macular finding. In conclusion, although 2 mg/0.05 mL ganciclovir is frequently used for intravitreal injection and is considered a safe dose for the treatment of ARN,4 ganciclovir retinal toxicity should be disclosed and fully discussed with patients before administering an intravitreal injection.

Outcome of pediatric patients with lymphoma following stem cell transplant: a single institution report

Abstract Hematopoietic stem cell transplant (HSCT) is recommended for pediatric patients with relapsed/refractory lymphoma even though the evidence for this is limited. We retrospectively reviewed records of 57 patients (29 Hodgkin lymphoma [HL], 28 non-Hodgkin lymphoma [NHL]) who underwent HSCT between 1995 and 2012. All demonstrated chemoresponsiveness prior to HSCT and 44 patients had a complete response. All underwent myeloablative conditioning, 38 chemotherapy-based and 19 total body irradiation-based. Forty-one patients received autologous and 16 allogeneic HSCT. Twelve (21%) died within 100 days post-HSCT, and 25 patients relapsed at a median of 1.6 months post-HSCT. Three patients developed second malignant neoplasms. Five-year overall survival (OS) was 50.5% and event-free survival (EFS) was 43.4%. Outcomes for HL were significantly better than those for NHL (OS 61.9% vs. 38.7% [p = 0.005] and EFS 60.4% vs. 26% [p = 0.008]). In summary, approximately half of all pediatric patients with lymphoma who failed first-line therapy and demonstrated chemosensitivity to second-line therapy can be salvaged with HSCT.

Treatment of a clinically determined lower-risk stage III non-lymphoblastic Non-Hodgkin lymphoma with less intensive therapy does not impact negatively on outcome.

Stage III NHL was divided into lower‐risk (LR) or high‐risk (HR) groups. Results of treatment were retrospectively reviewed for patients between 1993 through 2000. An intensive multiagent protocol was used for IIIHR, and a CHOP‐based, milder treatment for IIILR. Most LR therapy was outpatient, while treatment for HR patients was primarily inpatient. Five year EFS and OS for HR (n = 29) and LR (n = 23) groups was 86.2% and 95.6% (P = 0.26), and 93.1% and 100%, respectively (P = 0.4). LR had less toxicity. While these results need prospective confirmation, the data shows that less intensive therapy of a LR group of stage III NHL may not impact negatively on outcome. Pediatr Blood Cancer

Risk-adapted stratification for optimally intensive treatment assignment of pediatric patients with non-Hodgkin lymphoma is an effective strategy in developing countries

Pediatric patients with non‐Hodgkin lymphoma (NHL) in developing countries (DCs) present with greater tumor load even at lower stages and with comorbidities that impact therapy delivery. This causes toxic mortality with “standard” intensive protocols or recurrences with “gentler” treatment.

Pediatric Hodgkin Lymphoma: Making Progress

Hodgkin lymphoma (HL) is one of the more common cancers encountered among pediatric and adolescent patients; however, most HL occurs in adults, and children constitute only a small proportion of the total number of cases. Treatment outcomes for pediatric HL are excellent and current strategies focus on reduction of therapy-related toxicity while maintaining high survival. This has been achieved by identifying patient cohorts who are at a lower risk for relapse and can be successfully treated with reduced amounts of chemotherapeutic agents and radiation therapy. This identification has been assisted by better understanding of newer imaging modalities, in particular functional FDG–PET imaging. Patients who fail first-line therapy continue to have a chance of cure, and a variety of modalities are available for their treatment. New therapeutic agents, both traditional and biological, are under assessment.