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We report a case of stage IV primary mediastinal B-cell lymphoma in a 27-year-old young woman, who was refractory and chemoresistant to frontline conventional rituximab-based intensive chemotherapy and subsequent lines of conventional and immune checkpoint inhibitor-based therapies. She was successfully treated using a polatuzumab-based regimen and consolidated with an allogeneic haploidentical hematopoietic stem cell transplantation. She developed post-transplant large granular lymphocytosis that was managed conservatively. She is now relapse-free, 600 days post-transplant. The management of this patient provided several teaching points in the use of different modalities of immunotherapies in a hard-to-treat cancer and its related conditions. [ABSTRACT FROM AUTHOR]

Background: Diffuse large B-cell lymphoma (DLBCL) is the commonest subtype of lymphoma, standard CHOP was the treatment of choice, 42% of patients received rituximab, and 29% of patients were lost to follow-up during therapy, were reported in a study that collected retrospective data at 13 public and private hospitals for patients diagnosed with lymphoma between January 2005 and December 2009. The OncoCollect Registry was set up in 2017 to address the challenges in the collection of retrospective data through chart review, recording access to anthracycline and rituximab-based treatment, and to study outcomes and any improvement in the patient follow-up. Methodology: The OncoCollect Lymphoma group registry was set up at a national level with 9 participating centers. Lymphoma patients registered at these centers between 2011 and 2017 were included. The clinical features, prognostic stratification, associated comorbidities, response to first-line treatment, and 3-year outcomes of adult patients with DLBCL were analyzed. Results: Of the 5,886 lymphoma patients registered in the OncoCollect registry, 2,581 (44%) had DLBCL. A total of 1,961 were evaluable for frontline therapy. The median age at presentation was 57 years. Gender ratio was 1.6:1. At presentation, 43% were early stage, 70% had low and low intermediate IPI, 53% had extranodal disease, and 30.9% had one or more comorbidities (data available for 1,136 patients). The commonest extra nodal site was gastro-intestinal (23.98%) followed by head and neck (19.24%). The overall response rate was 79.29%. Complete remission was seen in 61.75%, partial response in 17.5%, stable disease in 4.3%, and progression in 7.9%. Patients who received anthracycline-based therapy (86.7%) and rituximab-based therapy (83.7%) had a 3-year event-free survival (EFS) of 69.67% and 68.48%, respectively. With a median follow-up of 33 months, the 3-year overall Survival (OS) and EFS were 75.37% and 66.58%, respectively. Conclusions: DLBCL remains the commonest (44%) lymphoma subtype and is curable with standard anthracycline- and rituximab-based therapies. The availability of rituximab has increased the proportion of patients receiving standard chemoimmunotherapy. [ABSTRACT FROM AUTHOR]

This case emphasizes that, with the availability of novel immunotherapy agents (Daratumumab), and repurposed use of bortezomib, a patient with HIV‐negative relapsed PBL can be treated successfully and consolidated with an allogeneic haploidentical hematopoietic cell transplantation. [ABSTRACT FROM AUTHOR]

Additionally, SP 18 sp F-fluorodeoxyglucose positron emission tomography (PET) and computed tomography (CT) have now become part of standard care in differentiating metabolic activity of the disease from fibrosis or necrosis [[3]]. Positron Emission Tomography (PET) Guided Therapy of Aggressive Lymphomas - Interim PET-Based Outcome Prediction and Treatment Changes in Patients with T Cell Lymphomas Participating in the PETAL Trial. [Extracted from the article]

Non-Hodgkin's lymphoma (NHL) is a common hematological malignancy. The age-adjusted incidence rates for NHL in men and women in India are 2.9/100,000 and 1.5/100,000, respectively. These are about one fourth of the incidence rates reported from Western Europe or North America. Within India, the incidence is several-fold higher in urban cancer registries compared to rural areas; the incidence being higher in metropolitan cities and Indian immigrants suggesting that urban lifestyles and economic progress may increase the cancer incidence. Compared to developed nations, the key differences in the presentation in India include: median age of 54 years (almost a decade less), higher male to female ratio, higher proportion of patients with B-symptoms (40-60 vs. 20-30%), poor ECOG performance status (= 2) at diagnosis (50 vs. 20-30%), higher frequency of diffuse large B-cell lymphomas (60-70 vs. <40%), lower frequency of follicular NHL (<20 vs. 30-40%) and T-cell type in 10-20 vs. <10%. The estimated mortality rate due to NHL is higher in India than in North America and Western Europe. Diagnostic and treatment delays, incorrect diagnosis and inappropriate or sub-optimal treatment may be possible reasons for the poor outcome. Any improvement in the outcomes for NHL in India will require a nationwide approach, e.g. creation of several regional and district-level centers with expertise in lymphoma management. Collection of data on patient- and diseaserelated characteristics, treatment outcome, development of infrastructure, centralized review of histopathology subtype, novel treatment protocols, rigorous follow-up, training of staff, and financial support towards treatment could be possible strategies to improve the outcome. [ABSTRACT FROM AUTHOR]

We compared the lomustine, cytarabine, cyclophosphamide and etoposide (LACE) and BCNU, etoposide, cytarabine, melphalan (BEAM) conditioning regimens for toxicity, engraftment kinetics, and efficacy in 139 patients undergoing autologous hematopoietic stem cell transplant for primary refractory or relapsed lymphoma. Ninety-two patients with Hodgkin lymphoma and 47 with non-Hodgkin lymphoma were enrolled. Seventy-five patients received LACE while 64 received BEAM. The incidence of grade 3-4 oral mucositis (9 vs 38%; P < 0.001) and parenteral nutrition requirement (32 vs 69%; P < 0.001) were significantly lower in the LACE cohort. The median days to myeloid (10 vs 11; P = 0.007) and platelet engraftment (13 vs 15; P = 0.026) were shorter for the LACE cohort. Transplant-related mortality in the LACE group was 9% compared to 13% in patients treated with BEAM (P = NS). The probability of overall survival (OS) and progression-free survival (PFS) at 5 years for entire cohort was 46 and 41%, respectively. Probability of OS (LACE 46% vs BEAM 47%; P = NS) and PFS (LACE 37% vs BEAM 47%; P = NS) at 5 years was comparable between two groups. We conclude that LACE has better toxicity profile compared to BEAM and results in similar long-term survival in primary refractory or relapsed lymphoma transplant. [ABSTRACT FROM AUTHOR]

Context: The distribution of various subtypes of lymphomas in India is different from other parts of the world. There is scarce multicentric data on the pattern and outcomes of lymphomas in India. Aims: The aim of this study is to evaluate the histopathological and the clinical pattern and treatment outcomes of lymphomas in India based on the retrospective data collected from a multicenter registry. Materials and Methods: Retrospective data was collected at 13 public and private hospitals in India for patients diagnosed with lymphoma between January 2005 and December 2009. The data collection was performed in the setting of a multicenter lymphoma registry Survival analyses were performed using the Kaplan-Meier method and compared using the logrank test. Results: Non-Hodgkin's lymphoma (NHL) constituted 83.17% and Hodgkin's lymphoma (HL) for 16.83% of the 1733 registered and analyzed cases. Diffuse large B cell lymphoma (DLBCL) was the most common NHL (55%) followed by follicular lymphoma (11%). CHOP was the most common chemotherapy regimen administered (84%) while rituximab was used in 42.7% of those with DLBCL. Survival analysis of treatment naïve DLBCL patients (n = 791) was performed. Of these, 29% were lost to follow-up, 20% with active disease. The median follow-up in surviving patients is 31 (range: 1-88) months. Median progression-free survival (PFS) and overall survival (OS) in DLBCL patients has not reached. There was no significant difference in median PFS (69 months vs. 61 months, P = 0.1341), but OS was significant not reached (NR) vs. NR, P = 0.0012) within international prognostic index high or intermediate subgroups. Rituximab use was associated with significantly prolonged PFS (NR vs. 82 months, P = 0.0123), but not OS (NR vs. NR, P = 0.2214). Cox regression analysis in treatment naïve DLBCL patients showed a performatnce status, stage and receipt of six or more cycles of chemotherapy to be significantly associated with OS and all of the preceding plus rituximab use significantly associated with PFS. Conclusions: Our analysis confirms previous reports of distribution of lymphoma subtypes in India and suggests that patients who are able to receive the full course of chemotherapy achieve a better outcome. This indicates the importance of ensuring compliance to treatment utilizing various measures including patient and family counseling. Prospective studies are required to confirm these findings. [ABSTRACT FROM AUTHOR]

We evaluated the diagnostic utility of flow cytometry immunophenotyping in bone marrow aspirates and peripheral blood, in the assessment of mature B-cell non-Hodgkin lymphoma (MBNHL). We analyzed 356 cases of MBNHL received for immunophenotyping over a 4 year period. All cases were reviewed, correlated with biopsy specimen (lymph node and splenectomy). Discrepant cases were re-evaluated. Common subtypes included chronic lymphocytic leukemia (CLL) (243 cases, 68.5%), follicular lymphoma (30 cases, 8.5%), mantle cell lymphoma (20 cases, 5.5%), splenic marginal zone lymphoma (18 cases, 5%), hairy cell leukemia (18 cases, 5%). CD5+/CD23+ had a high positive predictive value (PPV) for diagnosing CLL whereas CD5+/CD23- had a high negative predictive value (NPV) for diagnosing mantle-cell lymphoma (MCL). Limited panel of 9 antibodies mainly CD19, CD5, CD23, CD10, FMC7, kappa, lambda, CD3 and CD20 help diagnose more than 92% of cases of MBNHL. Minimal diagnostic panels become important in countries with limited resources. [ABSTRACT FROM AUTHOR]

Background: Haematolymphoid tumours other than plasmablastic lymphoma (PBL) may reveal plasmablastic differentiation with overlapping immunoreactivity causing diagnostic dilemma. Elaborate ancillary diagnostic techniques can make the process expensive, tedious and out of reach for pathology laboratory in a developing country. Methods: Out of 98 total cases of primary non-Hodgkin’s lymphoma and plasmacytoma of oral-sinonasal region recorded in our institute over 4 years, 39 cases showing varied plasmablastic differentiation were selected. Morphological and immunohistochemical criteria were applied to identify minimum diagnostic criteria for PBL. Human Immunodeficiency Virus (HIV) correlation and Epstein–Barr virus expressed RNA (EBER) in-situ hybridisation studies were also performed. Results: Minimum morphological criteria required to diagnose PBL were: (1) predominant population of plasmablasts which are large monomorphic cells with high nuclear-cytoplasmic ratio, moderate amount of amphophilic cytoplasm and round nucleus with prominent central nucleolus, (2) high mitotic and/or apoptotic index and (3) absence of neoplastic plasma cells in the background. Essential diagnostic immunophenotype consisted of CD20 negativity, LCA +/−, CD138/VS38c diffuse positivity, light chain restriction and high MIB-1 index (>60 %). Twenty-five of the total 32 PBL cases thus identified, involved oral cavity. Of these, 84% affected gingivo-buccal complex. Twenty-eight cases were HIV positive. EBER positivity confirmed the diagnosis in all the HIV-negative cases. Conclusions: A triad of ‘rapidly growing lesion with predilection for oral mucosa, classical plasmablastic morphology and limited immunohistochemical panel’ can render a reliable diagnosis of PBL, irrespective of HIV and EBV status, especially in developing countries with limited resources. [ABSTRACT FROM AUTHOR]

The article presents information on a study which evaluated the prognostic factors and treatment outcome of Indian patients with primary diffuse large B-cell lymphoma (DLBCL) of the tonsil treated at a single institution. The study included 121 patients with DLBCL of the tonsil treated at the Tata Memorial Hospital in Mumbai, India. The patients were treated from January 1990 to December 2002. It revealed some significant prognostic factors including disease-free survival and overall survival.

Primary orbital and primary breast lymphomas comprise very small subgroups of extranodal lymphomas. Clinical presentation at both these sites together is extremely rare. We describe a case of bilateral orbital and bilateral breast mucosa-associated lymphoid tissue (MALT) lymphomas with bilateral pre-auricular lymph nodal metastasis. The case history, staging and management for this unusual entity are discussed. [ABSTRACT FROM AUTHOR]

Primary female reproductive system lymphomas are distinctly uncommon. We report two cases with primary ovarian non Hodgkin's lymphoma (NHL) having unilateral involvement in one case while the other had bilateral ovarian involvement. Both cases initially presented with ovarian enlargement. The median size of the tumour was 5.8 × 5 cm². Histologically, by using the WHO classification, one lesion was classified as diffuse small B cell phenotype of intermediate grade and the other as diffuse high grade, B cell phenotype. On the basis of staging studies and clinical follow up, we conclude that both of the neoplasms in the report arose in the ovary. Both of the patients are disease free at 24 months and 6 months of follow up respectively, following excision and chemotherapy. We conclude that that the complete remission and failure free survival of patients with ovarian NHL treated with appropriate treatment appear to be similar to that of patients with extranodal NHL. [ABSTRACT FROM AUTHOR]

Solitary Extramedullary Plasmacytoma (EMP) is an uncommon neoplasm. When diagnosed, head and neck region is its most likely location. Rarely, it may occur in the retro-peritoneum. We report a 44year old man with solitary extramedullary plasmacytoma in the retro peritoneum (RPEMP). The patient did not show response to three cycles of VAD chemotherapy. Thereafter Surgical excision of the mass was performed successfully. This is probably the first case report from Indian subcontinent. [ABSTRACT FROM AUTHOR]