Primary intramedullary non-Hodgkin's lymphoma in an immunocompetent child.

STUDY DESIGN: Case report. OBJECTIVES: Primary intramedullary spinal cord lymphoma is a rare entity. Studies have shown that there is a recent increase in the number of patients regardless of the status of the immunity. High index of suspicion should be kept in all patients with intramedullary tumors. Multidisciplinary approach at the earliest is required for best outcomes. SETTING: Department of Neurosurgery, Medical Oncology, Pathology and Radiation oncology. Nizam's Institute of Medical Sciences, Hyderabad, India. METHODS: We describe the case of an 11-year-old boy who presented with paraparesis and sensory loss below T10 level. On imaging, the dorsal spine showed intramedullary lesion mimicking an astrocytoma. RESULTS: Surgical decompression of the tumor was done and histopathology showed non-Hodgkin's lymphoma, diffuse large B-cell type. There were no findings suggestive of congenital or acquired immunodeficiency. After complete staging evaluation, we instituted chemotherapy with modified DeAngelis protocol. At 2 years post treatment, he is in complete remission with near normal neurological status. CONCLUSIONS: Intramedullary spinal cord diffuse B-cell lymphoma in a pediatric age group is very rare and hence requires a high index of suspicion in patients presented with myelopathy. The outcomes are encouraging with current multidisciplinary approach.

Bortezomib in newly diagnosed patients with multiple myeloma: A retrospective analysis from a tertiary care center in India.

INTRODUCTION: Bortezomib is a novel proteasome inhibitor in myeloma. There is a paucity of data from India regarding the efficacy and tolerance to bortezomib. MATERIALS AND METHODS: All patients with newly diagnosed multiple myeloma from January 2008 to December 2011 treated with bortezomib as the first-line therapy were studied in a retrospective analysis. The primary end point was the overall rate of response. Secondary end points were the progression free survival (PFS), reversibility of renal compromise and safety of bortezomib. RESULTS: Our study included 41 patients with newly diagnosed myeloma. The overall response to bortezomib was 88.5% (complete response [CR] 31.4%, very good partial response 34.2%, partial response [PR] 22.8%). A renal response (CR renal, PR renal or Minimal Response renal combined) was documented 96.2% patients with initial renal impairment. The median time to the first renal response was 21 days. 17 patients (41.4%) had severe toxicity (Grade 3 and 4). Bortezomib induced peripheral neuropathy (BIPN) was the most common toxicity seen (53.6%) and the most common cause for discontinuation of therapy. At a median follow-up of 9 months, median PFS was not reached. DISCUSSION: The results obtained in our study are comparable with those of established studies on bortezomib. Our patient population has similar responses and renal reversibility patterns. However, they are at an increased susceptibility to BIPN, leading to discontinuation of therapy. CONCLUSION: Bortezomib as first-line therapy has a good efficacy and safety.

Imatinib mesylate as first-line therapy in patients with chronic myeloid leukemia in accelerated phase and blast phase: a retrospective analysis.

INTRODUCTION: Imatinib is a bcr-abl tyrosine kinase inhibitor which has revolutionized the treatment for chronic myeloid leukemia (CML). Even though there is much data on CML chronic phase, there is limited data on imatinib-naοve advanced phase CML. MATERIALS AND METHODS: We retrospectively analysed 90 patients with advanced phase CML (accelerated phase [AP]: 51 and blast crisis [BC]: 39), patients who received imatinib as frontline therapy. RESULTS: The median age of presentation in CML-AP and CML-BC were 32 years (12-61) and 39 years (8-59), respectively. Imatinib at 600 mg/day was initiated within 2 weeks of diagnosis. Median time to complete hematological response in both CML-AP and CML-BC was 3 months (CML-AP: 1-9 months and CML-BC: 1-14 months). At 6 months 30 (59%) CML-AP and 15 (38%) CML-BC patients achieved major cytogenetic response (MCyR), of them 24 (47%) and 10 (25.6%) being the complete cytogenetic response, respectively. At a median follow-up of 41 months, the median overall survival in CML-AP was 61 months, but in CML-BC it was 14 months. The median progression-free survival and event-free survival were 30 months and 23 months in CML-AP and 14 and 12 months in CML-BC, respectively. On univariate analysis, performance status (PS), spleen size, and MCyR predicted survival in AP, whereas in BC, platelet count, PS, and early MCyR were predictive. Non-hematologic and hematologic adverse events were observed in 80% and 60% of patients, respectively. Dose was reduced in 10% of patients for grade IV toxicity and interrupted in 30% for grade III toxicity. CONCLUSION: Front-line imatinib is an option in advanced phases of CML especially in CML-AP in low-resource countries, where stem cell transplantation and alternate TKIs are not available.