Tsukasaki K, Ikeda S, Murata K, Maeda T, Atogami S, Sohda H, Momita S, Jubashi T, Yamada Y, Mine M
Department of Hematology, Nagasaki University School of Medicine, Japan.
Leuk Res. 1993 Feb;17(2):157-66. doi: 10.1016/0145-2126(93)90061-o.
The acute and lymphoma types of adult T-cell leukemia/lymphoma (ATL) usually have a very poor prognosis, although some patients achieve long survival after chemotherapy. A total of 114 patients with these aggressive types of ATL were newly diagnosed at our institution from 1975 to 1989. By multivariate analysis, poor performance status and high serum creatine levels were associated with shortened survival. With combination chemotherapy, 20 patients achieved complete remission (CR), 53 achieved partial remission (PR) and 35 showed no response. Fifteen of the CR or PR patients survived for more than two years and all other patients survived for less than two years. As compared with short survivors (< 2 years) after remission, long survivors (> or = 2 years) after remission had a higher CR/PR ratio, a longer time until remission and a higher doxorubicin dose to achieve remission. Death due to causes other than the primary disease occurred in 18% of short survivors after remission and in 11.2% of nonresponders, but in none of the long survivors. Long survivors with acute ATL included 6 patients with CR and 5 patients with PR. All four lymphoma type ATL long survivors achieved CR. Monoclonal integration of HTLV-I provirus was detected in the peripheral blood mononuclear cells of all 3 PR long survivors with acute ATL studied, but was not detected in all 4 CR cases studied at remission. The minimum CD4/CD8 ratio of peripheral mononuclear cells at remission was < 1.0 in all acute ATL long survivors with CR, and was > 1.0 in all acute ATL long survivors with PR. Three out of six acute ATL long survivors with CR developed suspected viral infection just before achieving CR. Our findings show that in aggressive ATL the characteristics of remission are heterogeneous even among long survivors.
成人T细胞白血病/淋巴瘤(ATL)的急性型和淋巴瘤型通常预后很差,不过有些患者在化疗后可实现长期生存。1975年至1989年期间,我院共新诊断出114例这类侵袭性ATL患者。多因素分析显示,身体状况差和血清肌酸水平高与生存期缩短相关。采用联合化疗后,20例患者实现完全缓解(CR),53例实现部分缓解(PR),35例无反应。CR或PR患者中有15例存活超过两年,其他所有患者存活时间均不到两年。与缓解后生存期短(<2年)的患者相比,缓解后长期存活者(≥2年)的CR/PR比率更高、达到缓解所需时间更长且实现缓解所需的阿霉素剂量更高。缓解后因原发性疾病以外的原因死亡的情况在生存期短的患者中占18%,在无反应者中占11.2%,但在长期存活者中均未出现。急性ATL的长期存活者中有6例CR患者和5例PR患者。淋巴瘤型ATL的所有4例长期存活者均实现CR。在所研究的3例PR的急性ATL长期存活者的外周血单个核细胞中均检测到HTLV-I前病毒的单克隆整合,但在缓解期研究的所有4例CR病例中均未检测到。所有CR的急性ATL长期存活者缓解期外周单个核细胞的最低CD4/CD8比率<1.0,所有PR的急性ATL长期存活者的该比率>1.0。6例CR的急性ATL长期存活者中有3例在即将实现CR前出现疑似病毒感染。我们的研究结果表明,在侵袭性ATL中,即使在长期存活者中,缓解的特征也存在异质性。