Sapkota Sumina, Shaikh Hira
Gandaki Medical College, Pokhara
University of Cincinnati Medical Center
Non-Hodgkin lymphoma (NHL) is a neoplasm of the lymphoid tissues originating from B cell precursors, mature B cells, T cell precursors, and mature T cells. NHL comprises various subtypes, each with different epidemiologies, etiologies, immunophenotypic, genetic, clinical features, and response to therapy. It can be divided into 2 groups, 'indolent' and 'aggressive,' based on the disease's prognosis. The most common mature B cell neoplasms are Follicular lymphoma, Burkitt lymphoma, diffuse large B cell lymphoma, Mantle cell lymphoma, marginal zone lymphoma, and primary CNS lymphoma (see Mantle Cell Lymphoma). Adult T cell lymphoma and mycosis fungoides are the most common mature T cell lymphomas. The treatment of NHL varies greatly, depending on tumor stage, grade, type of lymphoma, and various patient factors (eg, symptoms, age, performance status). The natural history of these tumors shows significant variation. Indolent lymphomas present with waxing and waning lymphadenopathy for many years, whereas aggressive lymphomas have specific B symptoms such as weight loss, night sweats, and fever and can result in deaths within a few weeks if untreated. Lymphomas usually have indolent presentations, including follicular lymphoma, chronic lymphocytic leukemia/small lymphocytic lymphoma, and splenic marginal zone lymphoma. Aggressive lymphomas include diffuse large B cell lymphoma, Burkitt lymphoma, precursor B and T cell lymphoblastic leukemia/lymphoma, adult T cell leukemia/lymphoma, and other peripheral T cell lymphomas. Up to two-thirds of patients present with peripheral lymphadenopathy. Rashes on the skin, increased hypersensitivity reactions to insect bites, generalized fatigue, pruritus, malaise, fever of unknown origin, ascites, and effusions are less common presenting features. Approximately half of the patients develop extranodal disease (secondary extranodal disease) during their disease, while between 10 and 35 percent of patients have primary extranodal lymphoma at diagnosis. Primary gastrointestinal tract lymphoma may present with nausea and vomiting, aversion to food, weight loss, fullness of the abdomen, early satiety, and visceral obstruction-related symptoms. Patients may even present with features of acute perforation and gastrointestinal bleeding and, at times, with features of malabsorption syndrome. Primary central nervous system (CNS) lymphoma may present with headaches, spinal cord compression features, lethargy, focal neurologic deficits, seizures, and paralysis (see Central Nervous System Lymphoma, MRI).
非霍奇金淋巴瘤(NHL)是一种起源于B细胞前体、成熟B细胞、T细胞前体和成熟T细胞的淋巴组织肿瘤。NHL包括多种亚型,每种亚型具有不同的流行病学、病因、免疫表型、遗传学、临床特征及对治疗的反应。根据疾病的预后,它可分为“惰性”和“侵袭性”两组。最常见的成熟B细胞肿瘤是滤泡性淋巴瘤、伯基特淋巴瘤、弥漫性大B细胞淋巴瘤、套细胞淋巴瘤、边缘区淋巴瘤和原发性中枢神经系统淋巴瘤(见套细胞淋巴瘤)。成人T细胞淋巴瘤和蕈样肉芽肿是最常见的成熟T细胞淋巴瘤。NHL的治疗差异很大,取决于肿瘤分期、分级、淋巴瘤类型以及各种患者因素(如症状、年龄、体能状态)。这些肿瘤的自然病史差异显著。惰性淋巴瘤多年来表现为淋巴结肿大反复出现,而侵袭性淋巴瘤有特定的B症状,如体重减轻、盗汗和发热,如果不治疗,可能在几周内导致死亡。淋巴瘤通常有惰性表现,包括滤泡性淋巴瘤、慢性淋巴细胞白血病/小淋巴细胞淋巴瘤和脾边缘区淋巴瘤。侵袭性淋巴瘤包括弥漫性大B细胞淋巴瘤、伯基特淋巴瘤、前体B和T细胞淋巴母细胞白血病/淋巴瘤、成人T细胞白血病/淋巴瘤以及其他外周T细胞淋巴瘤。多达三分之二的患者表现为外周淋巴结肿大。皮肤皮疹、对蚊虫叮咬过敏反应增加、全身疲劳、瘙痒、不适、不明原因发热、腹水和积液是较少见的表现特征。约一半的患者在病程中会出现结外病变(继发性结外病变),而10%至35%的患者在诊断时患有原发性结外淋巴瘤。原发性胃肠道淋巴瘤可能表现为恶心、呕吐、厌食、体重减轻、腹部饱胀、早饱以及与内脏梗阻相关的症状。患者甚至可能出现急性穿孔和胃肠道出血的症状,有时还伴有吸收不良综合征的症状。原发性中枢神经系统(CNS)淋巴瘤可能表现为头痛、脊髓压迫症状、嗜睡、局灶性神经功能缺损、癫痫发作和瘫痪(见中枢神经系统淋巴瘤,MRI)。