PDF(Pubmed)
Abstract：
T-prolymphocytic leukemia (T-PLL) is a rare malignancy of mature T-cells with distinct clinical, cytomorphological, and molecular genetic features. The disease typically presents at an advanced stage, with marked leukocytosis, B symptoms, hepatosplenomegaly, and bone marrow failure. It usually follows an aggressive course from presentation, and the prognosis is often considered dismal; the median overall survival is less than one year with conventional chemotherapy. This case report describes a patient with T-PLL who, after an unusually protracted inactive phase, ultimately progressed to a highly invasive, organ-involving disease. After initial treatments failed, a novel treatment approach resulted in a significant response.
摘要：
T淋巴细胞白血病（T-PLL）是一种罕见的成熟T细胞恶性肿瘤，具有独特的临床特征，细胞形态学,和分子遗传特征。这种疾病通常出现在晚期，有明显的白细胞增多，B症状,肝脾肿大,骨髓衰竭.它通常遵循一个积极的过程，从演示，预后通常被认为是惨淡的;使用常规化疗的中位总生存期不到1年.本病例报告描述了一名患有T-PLL的患者，在异常持久的不活跃阶段之后，最终发展到高度侵入性，涉及器官的疾病。初步治疗失败后，一种新的治疗方法导致了显著的反应。
