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合并霍奇金病的慢性淋巴细胞白血病。对里德-施特恩贝格细胞起源的启示。

Chronic lymphocytic leukemia with coexistent Hodgkin's disease. Implications for the origin of the Reed-Sternberg cell.

作者信息

Williams J, Schned A, Cotelingam J D, Jaffe E S

机构信息

Department of Pathology, National Naval Medical Center, Bethesda, Maryland.

出版信息

Am J Surg Pathol. 1991 Jan;15(1):33-42. doi: 10.1097/00000478-199101000-00004.

DOI:10.1097/00000478-199101000-00004
PMID:1670753
Abstract

The association of chronic lymphocytic leukemia (CLL) and Hodgkin's disease has been controversial. Pleomorphic lymphoreticular cells resembling Reed-Sternberg cells have been observed in Richter's syndrome. Although most observers have favored the view that these cells are a component of a pleomorphic non-Hodgkin's lymphoma, some cases of histologically typical Hodgkin's disease have been described. We have studied two cases that appear to represent composite CLL and Hodgkin's disease, providing evidence for an interrelationship of these two disorders. Classic Reed-Sternberg cells and variants (RS-H) were seen in a background that was otherwise typical of CLL. Both patients initially presented with characteristic findings of CLL in the peripheral blood and bone marrow. The first patient was found to have RS-H cells within lymph nodes at initial presentation, and ultimately progressed to develop a disseminated lymphoma characteristic of Hodgkin's disease. In the second patient, RS-H cells were not discovered until 5 years later. Immunophenotypic studies confirmed these morphologic impressions. The predominant lymphocyte population had a phenotype consistent with B-cell CLL. By contrast, the RS-H cells were strongly positive for CD15 (Leu M1) with staining of the Golgi region and cell membrane. Additionally, the RS-H cells were surrounded by rosettes of lymphocytes that marked as T cells. In both of the patients, a small percentage of RS-H cells expressed positivity for the B-cell marker L-26, which may indicate an origin from the underlying CLL. These findings support a B-cell origin for the malignant cell in some cases of Hodgkin's disease and suggest that Hodgkin's disease in some patients may be related to or derived from a coexisting lymphoid malignancy.

摘要

慢性淋巴细胞白血病(CLL)与霍奇金病的关联一直存在争议。在 Richter 综合征中观察到了类似于里德-斯特恩伯格细胞的多形性淋巴网状细胞。尽管大多数观察者倾向于认为这些细胞是多形性非霍奇金淋巴瘤的一个组成部分,但也有一些组织学典型的霍奇金病病例被描述过。我们研究了两例似乎代表复合性 CLL 和霍奇金病的病例,为这两种疾病的相互关系提供了证据。在以 CLL 典型表现为主的背景中可见经典的里德-斯特恩伯格细胞及其变异型(RS-H)。两名患者最初在外周血和骨髓中均表现出 CLL 的特征性表现。首例患者在初次就诊时在淋巴结中发现有 RS-H 细胞,最终进展为播散性淋巴瘤,具有霍奇金病的特征。在第二例患者中,直到 5 年后才发现 RS-H 细胞。免疫表型研究证实了这些形态学表现。主要的淋巴细胞群体具有与 B 细胞 CLL 一致的表型。相比之下,RS-H 细胞对 CD15(Leu M1)呈强阳性,高尔基体区域和细胞膜均有染色。此外,RS-H 细胞被标记为 T 细胞的淋巴细胞玫瑰花环所环绕。在两名患者中,一小部分 RS-H 细胞对 B 细胞标志物 L-26 呈阳性表达,这可能表明其起源于潜在的 CLL。这些发现支持了某些霍奇金病病例中恶性细胞的 B 细胞起源,并提示某些患者的霍奇金病可能与共存的淋巴恶性肿瘤有关或源于此。

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