Slack Graham W, Ferry Judith A, Hasserjian Robert P, Sohani Aliyah R, Longtine Janina A, Harris Nancy L, Zukerberg Lawrence R
The James Homer Wright Pathology Laboratories, Massachusetts General Hospital, Boston, Massachusetts 02114, USA.
Leuk Lymphoma. 2009 Jun;50(6):937-43. doi: 10.1080/10428190902930488.
Lymphocyte depleted classical Hodgkin lymphoma (LDHL) is a vanishing category of classical Hodgkin lymphoma (CHL); many cases previously placed in this category are now recognised as diffuse large B-cell lymphoma (DLBCL), anaplastic large-cell lymphoma (ALCL), or nodular sclerosis CHL with lymphocyte depletion. In addition, the recent recognition of high grade B-cell lymphomas intermediate between DLBCL and CHL (grey-zone lymphomas) raises the question of whether LDHL exists at all as a category of CHL. We studied eight cases that fulfilled diagnostic criteria of LDHL according to the 2008 WHO Classification. The cases involved lymph nodes (7 cases) and pleura (1 case) from four males and four females (age 30-71 years; median 62 years). All tumors contained numerous Hodgkin-Reed-Sternberg (HRS) cells, fibroblasts and histiocytes and scattered lymphocytes. In three cases the tumors had a more diffuse fibrotic appearance, while in five cases they appeared reticular and anaplastic. Neoplastic cells in all cases expressed CD30, CD15, fascin, weak PAX5 and MUM-1 and lacked CD45, Alk-1, EMA, CD3, CD68, Mart-1 and cytokeratin. Oct.2 and/or Bob-1 were expressed in all cases. Two cases variably expressed CD20 but were CD79a negative. Four cases were positive for EBV. All the four cases with adequate DNA had clonally rearranged IGH genes. The combined morphologic, immunophenotypic and molecular genetic features of this group of cases distinguish LDHL from other disease entities, including grey-zone lymphomas.
淋巴细胞消减型经典型霍奇金淋巴瘤（LDHL）是经典型霍奇金淋巴瘤（CHL）中一种逐渐消失的类型；许多先前归为此类的病例现在被认为是弥漫性大B细胞淋巴瘤（DLBCL）、间变性大细胞淋巴瘤（ALCL）或伴有淋巴细胞消减的结节硬化型CHL。此外，最近对介于DLBCL和CHL之间的高级别B细胞淋巴瘤（灰色地带淋巴瘤）的认识，引发了LDHL是否作为CHL的一个类型而存在的问题。我们研究了8例根据2008年世界卫生组织分类符合LDHL诊断标准的病例。这些病例包括4名男性和4名女性（年龄30 - 71岁；中位年龄62岁）的淋巴结（7例）和胸膜（1例）。所有肿瘤均含有大量霍奇金 - 里德 - 斯腾伯格（HRS）细胞、成纤维细胞和组织细胞以及散在的淋巴细胞。3例肿瘤表现为更弥漫的纤维化外观，而5例表现为网状和间变性。所有病例中的肿瘤细胞均表达CD30、CD15、fascin、弱PAX5和MUM - 1，且缺乏CD45、Alk - 1、EMA、CD3、CD68、Mart - 1和细胞角蛋白。所有病例均表达Oct.2和/或Bob - 1。2例病例可变表达CD20但CD79a阴性。4例病例EBV阳性。所有4例有足够DNA的病例均有克隆性重排的IGH基因。这组病例的形态学、免疫表型和分子遗传学特征组合将LDHL与其他疾病实体区分开来，包括灰色地带淋巴瘤。
