Kawamoto Keisuke, Miyoshi Hiroaki, Yanagida Eriko, Yoshida Noriaki, Kiyasu Junichi, Kozai Yasuji, Morikita Tatsuma, Kato Takeharu, Suzushima Hitoshi, Tamura Shinobu, Muta Tsuyoshi, Kato Koji, Eto Tetsuya, Seki Ritsuko, Nagafuji Koji, Sone Hirohito, Takizawa Jun, Seto Masao, Ohshima Koichi
Department of Pathology, School of Medicine, Kurume University, Kurume, Japan.
Department of Hematology, Endocrinology, and Metabolism, Faculty of Medicine, Niigata University, Niigata, Japan.
Eur J Haematol. 2017 May;98(5):459-466. doi: 10.1111/ejh.12856. Epub 2017 Mar 3.
T-cell prolymphocytic leukemia (T-PLL) is a very rare, aggressive T-cell neoplasm. Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) is also a highly aggressive lymphoma. These two diseases can often be confused with each other; therefore, we aimed to determine the clinical and pathological differences between T-PLL and PTCL-NOS.
We analyzed 15 T-PLL and 91 PTCL-NOS patients and also compared clinical features between T-PLL and PTCL-NOS with leukemic presentation. Peripheral blood images and biopsy specimens were analyzed, and treatment responses were determined via imaging modalities. The clinicopathological characteristics were statistically compared.
T-PLL cells were smaller in size than those of PTCL-NOS with leukemic presentation (P=.0068); moreover, PTCL-NOS cells with leukemic presentation were smaller than those of PTCL-NOS without leukemic presentation (P=.0017). Immunophenotypic patterns in T-PLL and PTCL-NOS were similar. Five-year overall survival rates of T-PLL and all PTCL-NOS patients were 57.5% and 36.8%, respectively. No significant differences were found in clinical manifestations or prognoses; T-PLL and PTCL-NOS with leukemic presentation had essentially equivalent characteristics.
T-PLL and PTCL-NOS may share common biological and clinical characteristics in Japanese patients.
T 细胞原淋巴细胞白血病(T-PLL)是一种非常罕见的侵袭性 T 细胞肿瘤。外周 T 细胞淋巴瘤,非特指型(PTCL-NOS)也是一种高度侵袭性淋巴瘤。这两种疾病常常相互混淆;因此,我们旨在确定 T-PLL 与 PTCL-NOS 之间的临床和病理差异。
我们分析了 15 例 T-PLL 患者和 91 例 PTCL-NOS 患者,并比较了 T-PLL 与有白血病表现的 PTCL-NOS 之间的临床特征。分析外周血图像和活检标本,并通过影像学方法确定治疗反应。对临床病理特征进行统计学比较。
T-PLL 细胞的大小比有白血病表现的 PTCL-NOS 细胞小(P = 0.0068);此外,有白血病表现的 PTCL-NOS 细胞比无白血病表现的 PTCL-NOS 细胞小(P = 0.0017)。T-PLL 和 PTCL-NOS 的免疫表型模式相似。T-PLL 和所有 PTCL-NOS 患者的五年总生存率分别为 57.5%和 36.8%。在临床表现或预后方面未发现显著差异;有白血病表现的 T-PLL 和 PTCL-NOS 具有基本相同的特征。
在日本患者中,T-PLL 和 PTCL-NOS 可能具有共同的生物学和临床特征。