Várkonyi J, Zalatnai A, Timár J, Matolcsi A, Falus A, Bencsáth M, László V, Pócsik E, Kotlán B, Császár A
3rd Department of Internal Medicine, Semmelweis University of Medicine, Budapest, Hungary.
Acta Haematol. 2000;103(2):116-21. doi: 10.1159/000041031.
We describe a patient presenting with B cell chronic lymphocytic leukemia (B-CLL) who subsequently developed cutaneous infiltrates. Specimens of the blood, bone marrow and cutaneous infiltrations all showed the same heavy-chain gene rearrangement. Following failure of conventional chemotherapy, and in view of the similarity of the disease to cutaneous T cell lymphoma, interferon-alpha therapy was employed with satisfactory results. Introduction of this cytokine to the therapeutic modalities for secondary cutaneous B-CLL would hopefully change the poor outcome of this entity, or at least could produce a better quality of life. Loss of histidine decarboxylase activity in the infiltrating cells - in contrast to circulating lymphocytes - may be associated with the transformation of B-CLL to a more aggressive infiltrative form, offering a possible explanation for tissue invasiveness. The changing character of the disease raises the possibility of a second mutational event in the course of B-CLL.
我们描述了一名患有B细胞慢性淋巴细胞白血病(B-CLL)的患者,该患者随后出现了皮肤浸润。血液、骨髓和皮肤浸润的标本均显示出相同的重链基因重排。在传统化疗失败后,鉴于该疾病与皮肤T细胞淋巴瘤的相似性,采用了α干扰素治疗,结果令人满意。将这种细胞因子引入继发性皮肤B-CLL的治疗模式有望改变该疾病实体的不良预后,或者至少可以提高生活质量。与循环淋巴细胞相比,浸润细胞中组氨酸脱羧酶活性的丧失可能与B-CLL转变为更具侵袭性的浸润形式有关,这为组织侵袭性提供了一种可能的解释。疾病特征的变化增加了B-CLL病程中发生第二次突变事件的可能性。