Prochorec-Sobieszek Monika, Majewski Mirosław, Sikorska Anna, Centkowski Piotr, Tajer Joanna, Lampka-Wojciechowska Elzbieta, Rymkiewicz Grzegorz, Konopka Lech, Meder Janusz, Warzocha Krzysztof, Maryniak Renata K
Department of Pathomorphology, Institute of Hematology and Blood Transfusion, Warszawa.
Pol J Pathol. 2006;57(2):63-70.
During the course of lymphoma, a clinically more aggressive process with different morphology may develop, referred to as lymphoma transformation. Clonal relationship and pathogenic mechanism of this process are widely debated. The aim of the study was to evaluate morphology, immunophenotype (including EBV status) and clonal relationship in nine cases of lymphoma transformation. Among the six patients with low grade B-cell lymphomas three transformed into high grade B-cell lymphomas (two into diffuse large B-cell lymphoma, one into Burkitt lymphoma) and three into Hodgkin lymphoma. Three other patients with Hodgkin lymphoma presented with transformation into diffuse large B-cell lymphoma in two patients and peripheral T-cell lymphoma in one patient. In all cases there was a sudden clinical change as well as change in morphology and phenotype. In five of the nine patients studied EBV-LMP1 was demonstrated by immunohistochemistry in large transformed lymphoma cells. In two cases molecular studies revealed a different pattern of immunoglobulin gene rearrangement in the large transformed cells as compared to the small cells of primary indolent lymphoma. Thus, they represented secondary, arising de novo neoplasm.
在淋巴瘤病程中，可能会出现一种临床侵袭性更强、形态不同的过程，称为淋巴瘤转化。该过程的克隆关系和致病机制存在广泛争议。本研究的目的是评估9例淋巴瘤转化病例的形态学、免疫表型（包括EBV状态）和克隆关系。在6例低度B细胞淋巴瘤患者中，3例转化为高度B细胞淋巴瘤（2例转化为弥漫性大B细胞淋巴瘤，1例转化为伯基特淋巴瘤），3例转化为霍奇金淋巴瘤。另外3例霍奇金淋巴瘤患者中，2例转化为弥漫性大B细胞淋巴瘤，1例转化为外周T细胞淋巴瘤。所有病例均有临床突然变化以及形态和表型改变。在研究的9例患者中，5例经免疫组化证实大的转化淋巴瘤细胞中存在EBV-LMP1。2例分子研究显示，与原发性惰性淋巴瘤的小细胞相比，大的转化细胞中免疫球蛋白基因重排模式不同。因此，它们代表继发性的、新发的肿瘤。
