Nai G A, Cabello-Inchausti B, Suster S
Department of Pathology and Laboratory Medicine, Mount Sinai Medical Center, Miami Beach, FL, USA.
Pathol Res Pract. 1998;194(7):517-22. doi: 10.1016/S0344-0338(98)80122-2.
A case is presented of CD30+ anaplastic large cell lymphoma of the spleen. The patient, a 61 year old woman with a history of chronic lymphocytic leukemia (CLL) was seen for the sudden development of splenomegaly with thrombocytopenia. A splenectomy was performed which showed massive replacement of the spleen by a population of large atypical lymphoid cells showing bizarre nuclear forms and multinucleated tumor cells reminiscent of Reed-Sternberg cells. Immunohistochemical studies showed strong membrane and dot-like paranuclear positivity in the majority of the atypical cells for CD30, with coexpression in many of the cells for CD15. Additionally, the cells also strongly reacted with CD3, UCHL-1, EMA and LCA. The present case illustrates an unusual variant of anaplastic (CD30+) large cell lymphoma sharing histologic and immunophenotypic features that overlap with those of Hodgkin's disease. The history in this patient of CLL with sudden development of splenomegaly raises the possibility of transformation of CLL into a high-grade lymphoma (Richter's syndrome). The possible pathogenetic implications of this phenomenon are discussed.
本文报告一例脾脏CD30+间变性大细胞淋巴瘤。患者为一名61岁女性，有慢性淋巴细胞白血病（CLL）病史，因突然出现脾肿大伴血小板减少前来就诊。行脾切除术，结果显示脾脏被一群大的非典型淋巴细胞大量取代，这些细胞呈现奇异的核形态以及多核肿瘤细胞，让人联想到里德-施特恩伯格细胞。免疫组织化学研究显示，大多数非典型细胞的细胞膜及核旁呈点状强阳性表达CD30，许多细胞同时表达CD15。此外，这些细胞还与CD3、UCHL-1、EMA和LCA呈强反应。本例说明了间变性（CD30+）大细胞淋巴瘤的一种不寻常变异型，其组织学和免疫表型特征与霍奇金病重叠。该患者有CLL病史且突然出现脾肿大，提示CLL转化为高级别淋巴瘤（里氏综合征）的可能性。本文讨论了这一现象可能的发病机制。
