背景和目的:成人T细胞白血病/淋巴瘤(ATLL)是一种与人类I型T细胞淋巴细胞病毒(HTLV-1)相关的高度侵袭性T细胞淋巴增殖性疾病。ATLL是一种罕见的疾病,在HTLV-1流行区更常见,罗马尼亚就是其中之一。尽管治疗进展,预后仍然令人沮丧。我们的目的是描述临床,生物,罗马尼亚侵袭型ATLL患者的生存结局特征。材料和方法:我们报告了前瞻性的数据,观察,以及过去12年在我们中心诊断为淋巴瘤和急性类型ATLL的所有20例患者的单中心研究。数据来自患者的医疗记录。结果:淋巴瘤型ATLL(60%)比急性型ATLL(40%)更常见。诊断时的中位年龄为40.5岁,大多数患者是女性。实验室数据显示,急性和淋巴瘤型ATLL之间存在显着差异,即,急性型ATLL患者白细胞(p=0.02)和淋巴细胞计数(p=0.02)和校正钙水平(p=0.001)较高。所有患者均接受化疗,只有两个人接受了同种异体干细胞移植。只有六名患者对化疗有完全或部分反应,主要是淋巴瘤型的.所有患者的中位生存期为6.37个月,淋巴瘤型ATLL(8.16个月)的生存率高于急性型(3.60个月)。正常钙水平(p=0.011),尿酸(p=0.005),BUN评分(p=0.000),JCOG-PI中度风险(p=0.038),获得完全或部分缓解(p=0.037)与更高的生存率相关。结论:罗马尼亚患者的侵袭型ATLL表现出明显的特征,包括诊断时年龄较小,女性占主导地位,与目前报道的数据相比,淋巴瘤型ATLL的发病率更高。存活率仍然很低,所有亚型的中位生存期不到一年。
Background and Objectives: Adult T-cell leukemia/
lymphoma (ATLL) is a highly aggressive T-cell lymphoproliferative disease associated with the human T-cell lymphotropic virus type I (HTLV-1). ATLL is a rare disease, found more frequently in HTLV-1-endemic areas, Romania being one of them. Despite treatment advances, the prognosis remains dismal. We aimed to describe the clinical, biological, and survival outcome features of Romanian patients with aggressive-type ATLL. Materials and Methods: We report the data of a prospective, observational, and unicentric study of all 20 patients diagnosed with lymphoma and acute types of ATLL at our center over the past 12 years. Data were collected from the patients\' medical records. Results:
Lymphoma-type ATLL (60%) was more common than acute-type ATLL (40%). Median age at diagnosis was 40.5 years, and most patients were female. Laboratory data revealed significant differences between acute and
lymphoma-type ATLL, namely, higher leukocyte (p = 0.02) and lymphocyte counts (p = 0.02) and higher levels of corrected calcium (p = 0.001) in acute-type ATLL. All patients received chemotherapy, and only two underwent allogeneic stem cell transplantation. Only six patients obtained a complete or partial response to chemotherapy, mostly the
lymphoma-type ones. The median survival for all patients was 6.37 months, with higher survival in the
lymphoma-type ATLL (8.16 months) than in the acute-type (3.60 months). Normal calcium levels (p = 0.011), uric acid (p = 0.005), BUN score (p = 0.000), JCOG-PI moderate risk (p = 0.038), and obtaining complete or partial response (p = 0.037) were associated with higher survival. Conclusion: Aggressive-type ATLL among Romanian patients presents distinct characteristics, including younger age at diagnosis, female predominance, and higher incidence of lymphoma-type ATLL compared to currently reported data. Survival remains very low, with all subtypes experiencing a median survival of less than one year.