van der Kwast T H, van Dongen J J, Michiels J J, Hooijkaas H, Kappers M C, Hagemeijer A
Department of Pathology, Erasmus University, Rotterdam, The Netherlands.
Leukemia. 1991 Jan;5(1):78-82.
A 19-year-old man presented with cutaneous, mediastinal and intrapleural localization of a T-lymphoblastic non-Hodgkin's lymphoma (NHL) of immature phenotype. Two weeks after mediastinal irradiation the T-lymphoblasts had disappeared from the pleural effusion, but a clonal monocytic cell population was detected, as documented by immunological marker analysis and the presence of t(10;11), a cytogenetic aberration often associated with monocytic malignancies. Intensive chemotherapy induced a complete remission of the T-lymphoblastic NHL. However, the patient died from massive infiltration of lympho-hemopoietic tissue by cells with the morphology and immunological phenotype of macrophages. Southern blot analysis revealed the presence of a clonally rearranged immunoglobulin heavy chain (lgH) gene in tumorous tissue obtained at autopsy. The same clonally rearranged lgH was detectable in the post-irradiation pleural fluid 2 weeks after initial diagnosis. The observed germline configuration of T-cell receptor beta-genes and both lg light chain genes in this monoclonal proliferation provides additional evidence for the true histiocytic nature of the fatal disease. Therefore we conclude that a true histiocytic NHL with one rearranged lgH gene was most probably already present at initial diagnosis when the patient presented with the T-lymphoblastic NHL and that this true histiocytic NHL further developed despite the cytostatic treatment.
一名19岁男性患者，表现为具有不成熟表型的T淋巴细胞母细胞性非霍奇金淋巴瘤（NHL）的皮肤、纵隔和胸膜内定位。纵隔放疗两周后，T淋巴细胞母细胞从胸腔积液中消失，但检测到一个克隆性单核细胞群体，免疫标记分析及t(10;11)（一种常与单核细胞恶性肿瘤相关的细胞遗传学异常）的存在证实了这一点。强化化疗使T淋巴细胞母细胞性NHL完全缓解。然而，患者死于具有巨噬细胞形态和免疫表型的细胞对淋巴造血组织的大量浸润。Southern印迹分析显示，尸检获得的肿瘤组织中存在克隆性重排的免疫球蛋白重链（lgH）基因。初诊后2周，在放疗后的胸腔积液中可检测到相同的克隆性重排lgH。在这种单克隆增殖中观察到的T细胞受体β基因以及两个lg轻链基因的种系构型，为这种致命疾病的真正组织细胞性质提供了额外证据。因此我们得出结论，当患者表现为T淋巴细胞母细胞性NHL时，初诊时很可能已经存在一种具有一个重排lgH基因的真正组织细胞性NHL，并且尽管进行了细胞抑制治疗，这种真正组织细胞性NHL仍进一步发展。
