Introduction:Diffuse large B-cell lymphoma (DLBCL) is the most common subtype of non-Hodgkin's lymphoma. We conducted a retrospective study to analyze the clinicopathological characteristics, cell of origin, response to therapy, and the outcome of patients with DLBCL.Materials and Methods:This was a retrospective study which included all patients with DLBCL registered at our center, between May 1, 2013, and July 31, 2015. The data regarding demography, clinical presentation, histopathology, stage, prognostic index, treatment, and treatment-related outcome were collected from prospectively maintained clinical case records of the patients.Results:In the study, we included 267 patients. The median age is 49 (20–81) years with male: female ratio of 2:1. B symptoms were seen in 124 (45%) of patients. Early Stages (I and II) were seen in 130 (52%) patients, while advanced Stages (III and 1V) were seen in 119 (48%) patients. Bulky disease (>7.5 cm) was seen in 30% of cases, and bone marrow was involved in 12%. Extranodal involvement is present in 35% of cases. Cell of origin data was available in 160 (60%) of cases, of which 88 (55%) were germinal center and 72 (45%) were activated B cell in origin. The distribution according to the international prognostic index (IPI) was as follows: low risk 40%, intermediate risk 45%, and high risk in 15%. Rituximab was used in 45% of cases. The overall response rate was 84% with a complete response (CR) rate of 70.5%. The CR rates were better with RCHOP compared with CHOP (77% vs. 61.5%, P = 0.001) and good-risk IPI (83.3% vs. 65.2%, P < 0.001) compared with intermediate- and high-risk IPI. Median follow-up period was 24 months, and 2-year event-free survival (EFS) was 70%. The presence of B symptoms, high IPI, failure to attain CR, poor PS, and nonrituximab-based chemotherapy were significantly associated with lower EFS.Conclusions:This is the first study from India, which investigated the impact of chemotherapy with or without rituximab in context of cell of origin. Adding rituximab to CHOP showed better response rate and EFS irrespective of cell of origin.
Objective: Langerhans cell histiocytosis (LCH) is a rare disease affecting predominantly children and young adults but can be found in any age group. Diagnosis of LCH is often difficult and can be delayed because of its rarity. The present study highlights the cytomorphological features in a large cohort of cases. An accurate cytological diagnosis may avoid unnecessary biopsy and guide appropriate management.Method: Fourty seven (47) cases of LCH diagnosed on cytological material & fineneedle aspiration (FNA) over a period of 14 years (2003)(2004)(2005)(2006)(2007)(2008)(2009)(2010)(2011)(2012)(2013)(2014)(2015)(2016) were retrieved from the archives. The cytological smears were evaluated and microscopic findings collected by semi-quantitative assessment done by two different pathologists. Result:The age at the diagnosis of the patients ranged from 9 months to 28 years.The majority of cases were in the age group of 0-5 years. The most common site was head and neck region, which included cervical lymphadenopathy and scalp swelling.Two cases were diagnosed each from inguinal lymph node and bronchio-alveolar lavage (BAL). Cytological smears in the majority of the cases were moderate to highly cellular (58%) and showing abundant Langerhans cell in (72%) of cases. Areas of necrosis were seen in 38%, while 78% of cases showed giant cells. The majority of cases showed mild eosinophilia (61%), sparse lymphocytosis (83%) and mild neutrophilic infiltration (64%). There were 1-2 mitoses per 10 high power field in 12 cases (25.5%).No abnormal mitoses were identified. Conclusion: The presence of cells with features of Langerhans cells associated withthe expression of selected immunohistochemical markers allow the diagnosis of LCH on cytological samples, sparing more invasive procedure as a biopsy. K E Y W O R D S bony lytic lesion, fine needle aspiration cytology, histiocytosis, Langerhans cell histiocytosis, Langerhans cells, Langerin 414 | PHULWARE Et AL. 1 | INTRODUC TI ON Langerhans cell histiocytosis (LCH) is a rare disorder that arises due to abnormal clonal proliferation of Langerhans cells. 1,2 These cells belong to the family of dendritic cells and were first described by Paul Langerhans in 1868. 2 The disease has varied nomenclature depending on the location and distribution of these abnormal cells, namely, Hand-Schuller-Christian disease, eosinophilic granuloma, histiocytosis X and Letterer-Siwe disease. 3 The disease is common in childhood but may progress into adult life. LCH may affect one or multiple organs and tissues, such as bone, lung, skin, mucous membranes, lymph nodes, hypothalamus or posterior pituitary gland, liver and various soft tissues with a clinical course varying from localized diseases to more disseminated and aggressive forms. 4,5 The revised classification of histiocytes consist of five groups of diseases: (a) Langerhans-related; (b) cutaneous and mucocutaneous; (c) malignant histiocytoses; (d) Rosai-Dorfman disease (RDD); and (e) haemophagocytic lymphohistiocytosis and macrophages activation...
Purpose: Peripheral T-cell lymphoma (PTCL) is a heterogeneous group of non–Hodgkin lymphomas with aggressive clinical behavior. We performed comprehensive miRNA profiling in PTCLs and corresponding normal CD4+ Th1/2 and TFH-like polarized subsets to elucidate the role of miRNAs in T-cell lymphomagenesis. Experimental Design: We used nCounter (NanoString Inc) for miRNA profiling and validated using Taqman qRT-PCR (Applied Biosystems, Inc). Normal CD4+ T cells were polarized into effector Th subsets using signature cytokines, and miRNA significance was revealed using functional experiments. Results: Effector Th subsets showed distinct miRNA expression with corresponding transcription factor expression (e.g., BCL6/miR-19b, -106, -30d, -26b, in IL21-polarized; GATA3/miR-155, miR-337 in Th2-polarized; and TBX21/miR-181a, -331-3p in Th1-polarized cells). Integration of miRNA signatures suggested activation of TCR and PI3K signaling in IL21-polarized cells, ERK signaling in Th1-polarized cells, and AKT–mTOR signaling in Th2-polarized cells, validated at protein level. In neoplastic counterparts, distinctive miRNAs were identified and confirmed in an independent cohort. Integrative miRNA–mRNA analysis identified a decrease in target transcript abundance leading to deregulation of sphingolipid and Wnt signaling and epigenetic dysregulation in angioimmunoblastic T-cell lymphoma (AITL), while ERK, MAPK, and cell cycle were identified in PTCL subsets, and decreased target transcript abundance was validated in an independent cohort. Elevated expression of miRNAs (miR-126-3p, miR-145-5p) in AITL was associated with poor clinical outcome. In silico and experimental validation suggest two targets (miR-126→ SIPR2 and miR-145 → ROCK1) resulting in reduced RhoA-GTPase activity and T–B-cell interaction. Conclusions: Unique miRNAs and deregulated oncogenic pathways are associated with PTCL subtypes. Upregulated miRNA-126-3p and miR-145-5p expression regulate RhoA-GTPase and inhibit T-cell migration, crucial for AITL pathobiology.
This is the largest study from south Asia on advanced stage pediatric B-NHL and it suggests undernourishment, poor performance status and gender bias to be unique features at presentation. Although, outcomes are comparable with other data from resource-challenged nations, yet they are 15-20% inferior than trial data from other developed countries. Further, poor performance status, female sex and urban residence for poor outcome were identified as unique prognostic factors.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.