马来西亚弥漫性大b细胞淋巴瘤研究综述

IF 0.6 4区 医学 Q4 PATHOLOGY Malaysian Journal of Pathology Pub Date : 2023-04-01
K G Lim, A Sumera, I A S Burud, S P Venkateswaran
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引用次数: 0

摘要

淋巴瘤在世界范围内普遍存在,在马来西亚是一种常见的恶性肿瘤。弥漫性大b细胞淋巴瘤(DLBCL)是b细胞淋巴瘤最常见的亚型,占所有b细胞淋巴瘤的54% ~ 65%,占所有恶性淋巴瘤的39% ~ 57%。然而,DLBCL是一组异质性的疾病,具有不同的临床表现、生物学和对治疗的反应。在了解DLBCL的遗传景观和分子特征方面的最新进展已经确定了对化学免疫治疗结果不佳的高风险亚群,这些亚群正在各种临床试验中积极研究。C-MYC是位于染色体8q24上的原癌基因。10%到15%的新诊断的DLBCL患者有潜在的MYC癌基因重排,导致细胞存活和增殖失调。这些病例中约有一半还携带抗凋亡原癌基因BCL2和/或其转录抑制因子BCL6的重排。在文献中发现了超过20例具有显著特征的马来西亚DLBCL病例报告,此外还有一些广泛的病例系列,并包括在本综述中。R-CHOP仍然是主要的治疗方法,可以帮助近90%的有限期患者和高达60%的晚期患者实现长期疾病控制。本综述收集了马来西亚报道DLBCL的所有52项研究,并总结了基本方面,包括患病率、亚型、预后标志物、临床表现特征和可用病例的有限结果。
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Diffuse large B-cell lymphoma research in Malaysia: A review.

Lymphomas are prevalent worldwide and a common malignancy reported in Malaysia. Diffuse large B-cell lymphoma (DLBCL) is the most common subtype of B-cell lymphomas accounting for 54% to 65% of all B-cell lymphomas and 39% to 57% of all malignant lymphomas. However, DLBCL comprises a heterogeneous group of diseases with different clinical presentations, biology and response to treatment. Recent advances in understanding the genetic landscape and molecular features of DLBCL have identified high-risk subsets with poor outcomes to chemo-immunotherapy that are actively being studied in various clinical trials. C-MYC is a proto-oncogene located in chromosome 8q24. 10 to 15 % of patients with newly diagnosed DLBCL have an underlying rearrangement of the MYC oncogene, resulting in dysregulated cellular survival and proliferation. Approximately half of these cases also carry a rearrangement of the anti-apoptotic proto-oncogene BCL2 and/or its transcription repressor BCL6. Over 20 case reports of DLBCL cases with notable features in Malaysia have found in the literature, in addition to a few extensive case series and included in this review. R-CHOP remains the mainstay of therapy and can help achieve control of long-term disease in nearly 90% of patients presenting with limited-stage and in up to 60% of those presenting with advanced stages. This review captures all 52 studies that reported DLBCL in Malaysia and summarises the essential aspects, including prevalence, subtype, prognostic markers clinical features in presentation and limited outcomes of cases when available.

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来源期刊
CiteScore
3.60
自引率
5.60%
发文量
34
期刊介绍: The Malaysian Journal of Pathology is the official journal of the College of Pathologists, Academy of Medicine Malaysia. The primary purpose of The Journal is to publish the results of study and research in Pathology, especially those that have particular relevance to human disease occurring in Malaysia and other countries in this region. The term PATHOLOGY will be interpreted in its broadest sense to include Chemical Pathology, Cytology, Experimental Pathology, Forensic Pathology, Haematology, Histopathology, Immunology, Medical Microbiology and Parasitology. The Journal aims to bring under one cover publications of regional interest embracing the various sub-specialities of Pathology. It is expected that the articles published would be of value not only to pathologists, but also to medical practitioners in search of a scientific basis for the problems encountered in their practice, and to those with an interest in diseases which occur in the tropics.
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