Clinical spectrum of lymphoproliferative diseases
Clinical spectrum of lymphoproliferative diseases
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淋巴增殖性疾病的临床谱
作者：
D. Galton;D. Catovsky;E. Wiltshaw
虽然高度多样化，临床表现的模式是一致的，越来越多的证据表明，大多数淋巴组织增生性疾病是单克隆增殖。恶性程度最高的，通常是分化较差的弥漫性淋巴细胞性或组织细胞性肿瘤，可能表现为局限于原发部位的局灶性肿瘤，常位于淋巴结部位。它们像癌一样通过局部浸润邻近组织转移，扩散到区域淋巴结，并产生离散的远端血源性沉积物。滤泡性淋巴瘤在其恶性阶段可能通过这种病理过程传播。分化良好的淋巴细胞性淋巴瘤的分布，通常表现为全身性分布，提示来自祖细胞的不同传播机制，其后代可能永远不会形成肿瘤块，但似乎通过与所涉及淋巴细胞类型相适应的生理再循环过程进行传播。淋巴细胞通过归巢机制优先集中在特定组织或器官：因此滤泡性淋巴瘤良性阶段的滤泡中心B细胞归巢淋巴结，在那里它们产生多个滤泡结构，而Sézary真菌病复合体的T细胞对皮肤具有亲和力。在弥漫性高分化淋巴细胞性淋巴瘤中，淋巴细胞回到淋巴结、脾和骨髓。恶性淋巴组织增生性疾病也可能发生在已经通过生理性传播而普遍化的单克隆中：例如特发性冷血凝素病中发生的淋巴瘤和a链疾病（免疫增殖性小肠疾病）中的多灶性肿瘤。
Although highly diverse, the patterns of clinical presentation are consistent with the growing evidence that most lymphoproliferative diseases are monoclonal proliferations. The most malignant, usually poorly differentiated diffuse lymphocytic or “histiocytic” tumors may present as focal tumors localized to a primary, often extranodal, site. They metastasize like carcinomas by local invasion of adjacent tissues, spread to regional lymph nodes, and production of discrete distant blood‐borne deposits. Follicular lymphoma in its malignant phase may disseminate by this pathological process. The distribution of the well‐differentiated lymphocytic lymphomas, usually generalized at presentation, suggests a different mechanism of dissemination from a progenitor cell whose descendants may never form a tumor mass but appear to disseminate by the physiological process of recirculation appropriate to the type of lymphocyte involved. The lymphocytes concentrate preferentially in particular tissues or organs by a homing mechanism: thus follicular center B cells of the benign phase of follicular lymphoma home to lymph nodes where they give rise to multiple follicular structures, while the T‐cells of the Sézary‐mycosis fungoides complex have an affinity for skin. In the diffuse well‐differentiated lymphocytic lymphomas, the lymphocytes home to the lymph nodes, spleen and bone‐marrow. Malignant lymphoproliferative disease can also arise in a monoclone already generalized by physiological dissemination: examples are lymphoma arising in idiopathic cold‐haemagglutinin disease, and multifocal tumors in a‐chain disease (immunoproliferative small intestinal disease).
影响因子：
11.2
作者：
P. Carbone;H. Kaplan;K. Musshoff;D. Smithers;M. Tubiana
通讯作者：
P. Carbone;H. Kaplan;K. Musshoff;D. Smithers;M. Tubiana