Kadin M E, Sako D, Berliner N, Franklin W, Woda B, Borowitz M, Ireland K, Schweid A, Herzog P, Lange B
Blood. 1986 Nov;68(5):1042-9.
We describe a large-cell lymphoma of activated lymphoid cells in six children and adolescents. The presenting clinical features of regressing skin lesions and peripheral lymphadenopathy, sinus infiltration of lymph nodes, and infrequent tumor cell erythrophagocytosis resulted in initial diagnoses of malignant or regressing atypical histiocytosis in five cases. Binucleate and multinucleate tumor cells, sometimes with prominent eosinophilic nucleoli, resembled Reed-Sternberg (RS) cells and occasionally were found in a cytoarchitectural milieu that was consistent with a diagnosis of Hodgkin's disease (HD). The tumor cells did in fact express the HD-associated antigen Ki-1, but unlike most types of HD, the RS-like cells expressed common leukocyte antigen and were negative for Leu-M1. A T cell origin for the malignant cells was demonstrated with monoclonal antibodies in two cases, by focal staining for nonspecific esterase in one case, and by rearrangement of the beta-chain genes for the T cell receptor in a fourth case. These studies provide further evidence that some cases previously interpreted as malignant or regressing atypical histiocytosis and some types of HD are actually T cell disorders.
我们描述了6名儿童和青少年的活化淋巴细胞大细胞淋巴瘤。其呈现出的临床特征为皮肤病变消退、外周淋巴结病、淋巴结窦浸润以及罕见的肿瘤细胞吞噬红细胞现象，这导致5例患者最初被诊断为恶性或消退性非典型组织细胞增多症。双核和多核肿瘤细胞，有时伴有明显的嗜酸性核仁，类似于里德-施特恩贝格（RS）细胞，偶尔在符合霍奇金病（HD）诊断的细胞结构环境中被发现。肿瘤细胞实际上表达了与HD相关的抗原Ki-1，但与大多数类型的HD不同，RS样细胞表达常见白细胞抗原且对Leu-M1呈阴性。在2例中通过单克隆抗体证实恶性细胞起源于T细胞，1例通过非特异性酯酶局灶性染色证实，第4例通过T细胞受体β链基因重排证实。这些研究进一步证明，一些先前被解释为恶性或消退性非典型组织细胞增多症的病例以及某些类型的HD实际上是T细胞疾病。
