结节性淋巴细胞为主的霍奇金淋巴瘤伴结节性硬化

S. El Hussein, Xiaoqiong Wang, Hong Fang, F. Jelloul, Wei Wang, S. Loghavi, F. Vega, R. Miranda, T. Muzzafar, J. Manning, J. Khoury, W. Burack, A. Evans, L. Medeiros
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摘要

结节性淋巴细胞为主的霍奇金淋巴瘤(NLPHL)具有不寻常的特征,包括一些可以在形态学上与经典霍奇金淋巴瘤(CHL)重叠的病例。在此,我们描述了12例NLPHL伴有纤维带和荚膜纤维化,部分类似于结节性硬化症(NS) CHL。12例中有7例含有reed - sternberg样细胞,进一步提示CHL,但所有病例背景中均缺乏相关的嗜酸性粒细胞和/或浆细胞。在该队列中,所有病例中,7例(58%)病例中存在所谓的D型(结节性t细胞丰富)区域,或5例(42%)病例中存在E型(弥漫性t细胞/丰富组织细胞)区域的混合模式。在这些病例中,大肿瘤细胞的免疫表型支持它们是NLPHL的淋巴细胞优势细胞,CD20、CD79a和OCT2阳性,CD15和CD30阴性。然而,PAX5在11例中有9例与CHL中的Hodgkin/Reed-Sternberg细胞相似。我们的结论是,一些NLPHL病例与纤维带和荚膜纤维化有关,部分类似于nschl。根据我们的经验,具有ns样特征的NLPHL发生在10%至15%的NLPHL病例中,并与变异模式(D和/或E)相关。此外,该队列中的所有患者在活检前均未接受治疗,这表明这些病例中突出的硬化是疾病生物学固有的。识别具有ns样特征的NLPHL进一步扩展了NLPHL的形态学谱,有助于避免误诊为CHL的可能。
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Nodular Lymphocyte–predominant Hodgkin Lymphoma With Nodular Sclerosis
Nodular lymphocyte–predominant Hodgkin lymphoma (NLPHL) with unusual features, including some that can overlap morphologically with classic Hodgkin lymphoma (CHL), have been described. Herein, we describe 12 cases of NLPHL with fibrous bands and capsular fibrosis resembling, in part, nodular sclerosis (NS) CHL. Seven of 12 cases harbored Reed-Sternberg–like cells, further suggestive of CHL, but all cases lacked associated eosinophils and/or plasma cells in the background. In this cohort, all cases had areas of so-called pattern D (nodular T-cell rich) as a sole component in 7 (58%) cases or as a hybrid pattern along with pattern E (diffuse T-cell/histiocyte-rich) in 5 (42%) cases. The immunophenotype of the large neoplastic cells in these cases supported their being lymphocyte predominant cells of NLPHL, positive for CD20, CD79a, and OCT2, and negative for CD15 and CD30. However, PAX5 was weak in 9 of 11 cases similar to Hodgkin/Reed-Sternberg cells in CHL. We conclude that some cases of NLPHL are associated with fibrous bands and capsular fibrosis and resemble, in part, NS CHL. In our experience, NLPHL with NS-like features occurs in 10% to 15% of cases of NLPHL and is associated with a variant pattern (D and/or E). In addition, all patients in this cohort were not treated before biopsy, suggesting that the prominent sclerosis in these cases is inherent to disease biology. Recognition of NLPHL with NS-like features further expands the morphologic spectrum of NLPHL and helps avoid potential misdiagnosis as CHL.
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