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恶性淋巴瘤,淋巴细胞母细胞性

Malignant lymphoma, lymphoblastic.

作者信息

Nathwani B N, Kim H, Rappaport H

出版信息

Cancer. 1976 Aug;38(2):964-83. doi: 10.1002/1097-0142(197608)38:2<964::aid-cncr2820380248>3.0.co;2-v.

Abstract

Among the malignant lymphomas of the diffuse, poorly differentiated lymphocytic type, a cytologically distinctive form can be recognized. It is composed of immature lymphoid cells that are indistinguishable from the cells of acute lymphoblastic leukemia (ALL). Although these neoplasms usually have been classified as malignant lymphoma, lymphoblastic type, they contain, in addition to lymphoblasts, prolymphocytes in varying proportions. On the basis of the nuclear morphology, malignant lymphoma of the lymphoblastic type, (MLLB) can be further divided into those with and those without convoluted nuclei. In our series both groups had the following clinical features in common: 1) frequent occurrence in children and adolescents; 2) clinical presentation with mediastinal masses in 50% of cases; 3) a high incidence of bone marrow and perpheral blood involvement during the course of the disease; and 4) rapid progression of the disease with a median survival of 8 months. Our observations indicate that nuclear convolutions are helpful but not essential for the recognition of a clinicopathologic entity which is histologically and cytologically characterized by 1) the immaturity of the lymphoid cells indistinguishable from the lymphoblasts and prolymphocytes of ALL and 2) a high mitotic index. Because of the frequency with which MLLB progresses into ALL, systemic therapy may be indicated even before this progression is hematologically evident. This indicates the need for morphologic recognition of this malignant lymphoma regardless of the presence of nuclear convolution, age of the patient, and site of presentation.

摘要

在弥漫性、低分化淋巴细胞型恶性淋巴瘤中,可识别出一种在细胞学上具有独特形态的类型。它由未成熟的淋巴细胞组成,这些细胞与急性淋巴细胞白血病(ALL)的细胞难以区分。尽管这些肿瘤通常被归类为淋巴母细胞型恶性淋巴瘤,但除了淋巴母细胞外,还含有不同比例的原淋巴细胞。根据核形态,淋巴母细胞型恶性淋巴瘤(MLLB)可进一步分为有核卷曲和无核卷曲的两类。在我们的病例系列中,这两组具有以下共同临床特征:1)常见于儿童和青少年;2)50%的病例临床表现为纵隔肿块;3)病程中骨髓和外周血受累发生率高;4)疾病进展迅速,中位生存期为8个月。我们的观察表明,核卷曲有助于识别一种临床病理实体,但并非必需,该实体在组织学和细胞学上的特征为:1)淋巴细胞不成熟,与ALL的淋巴母细胞和原淋巴细胞难以区分;2)有丝分裂指数高。由于MLLB进展为ALL的频率较高,即使在血液学上尚未明显出现这种进展之前,也可能需要进行全身治疗。这表明无论是否存在核卷曲、患者年龄和发病部位,都需要对这种恶性淋巴瘤进行形态学识别。

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