Trümper L, Matthaei-Maurer D U, Knauf W, Möller P
Medizinische Klinik und Poliklinik, Universität Heidelberg.
Klin Wochenschr. 1988 Aug 15;66(16):736-42. doi: 10.1007/BF01726417.
A 67-year-old woman suffering since 5 years from a so far nontreated B-CLL underwent hemithyroidectomy for a rapidly enlarging tumor. Histologically, the coincidence of a centroblastic lymphoma and thyroidal infiltration by the CLL was diagnosed. Immunohistology revealed typical immunoprofils for both, B-CLL and centroblastic lymphoma on the background of B cell differentiation antigens. The bitypical immunoglobulin light chain expression--lambda on the B-CLL cells and kappa on the centroblasts--suggested biclonality. This was confirmed by gene rearrangement analysis of peripheral leukemia cells and tumor tissue. Thus, the final diagnosis of a primary thyroidal lymphoma of the centroblastic type (stage IE) arising independently from a preexisting B-CLL was achieved. Consequently, the patient received local radiotherapy. In our opinion, the designation "Richter's Syndrome", readily applied in the literature, is inappropriate for this tumor constellation.
一名67岁女性,患B细胞慢性淋巴细胞白血病(B-CLL)5年,此前未接受过治疗,因肿瘤迅速增大接受了半甲状腺切除术。组织学检查诊断为中心母细胞性淋巴瘤与CLL甲状腺浸润并存。免疫组织化学在B细胞分化抗原背景下显示了B-CLL和中心母细胞性淋巴瘤的典型免疫表型。双型免疫球蛋白轻链表达——B-CLL细胞上为λ链,中心母细胞上为κ链——提示双克隆性。外周白血病细胞和肿瘤组织的基因重排分析证实了这一点。因此,最终诊断为独立于先前存在的B-CLL发生的原发性中心母细胞型甲状腺淋巴瘤(IE期)。因此,该患者接受了局部放疗。我们认为,文献中常用的“ Richter综合征”这一名称不适用于这种肿瘤情况。