Zhang Yan-ru, Wang Yan-ying, Li Zeng-jun, Yi Shu-hua, Feng Xiao-yan, Liu Wei, Qi Jun-yuan, Zou De-hui, Zhao Yao-zhong, Qiu Lu-gui
Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin 300020, China.
Zhonghua Yi Xue Za Zhi. 2012 Dec 11;92(46):3250-3.
To explore the clinical and laboratory characteristics and survival of Chinese patients with hairy cell leukemia (HCL).
A total of 30 HCL patients from August 1990 to March 2012 were retrospectively analyzed.
There were 22 cases with classical HCL (HCL-C) and 8 with variant HCL (HCL-V). Splenomegaly was the most common physical finding. Leukocytosis was found in all cases of HCL-V. But pancytopenia only accounted for 36.4% (8/22) in HCL-C. And 3/5 of HCL-V had abnormal chromosome karyotypes. Ribosomal-lamellae complexes (RLC) were found only in about 3/12 of HCL cases. Chemotherapy regimens including purine nucleoside analogues achieved a better complete remission (CR) rate than other regimens (3/4 vs 1/18, P = 0.012) in HCL-C. The median follow-up period was 27 (1 - 142) months. There was no follow-up loss. Eleven cases progressed and 6 died. The median overall survival (OS) was not reached. And the 1, 3, 6-year OS rates were 84%, 78% and 58% respectively. The median progression-free survival (PFS) was (63 ± 24) months and the 1, 2, 5-year PFS rates were 86%, 72% and 44% respectively. The median PFS of HCL-V was significant shorter than HCL-C ((23 ± 3) vs (78 ± 12) months, P = 0.014). In HCL-C group, fever (P = 0.038) and anemia (P = 0.000) at diagnosis were poor prognostic factors. But purine nucleoside analogues made no significant difference in PFS.
Pancytopenia is infrequent in Chinese HCL patients. And classical RLC is rare under electron microscope. Purine nucleoside analogues may achieve a better CR rate, but fail to improve PFS rate. As compared with HCL-C, HCL-V is common with genetic abnormalities and has a worse prognosis with a shorter PFS.
探讨中国毛细胞白血病（HCL）患者的临床和实验室特征及生存情况。
回顾性分析1990年8月至2012年3月期间共30例HCL患者。
22例为经典型HCL（HCL-C），8例为变异型HCL（HCL-V）。脾肿大是最常见的体格检查发现。所有HCL-V患者均有白细胞增多。但全血细胞减少在HCL-C中仅占36.4%（8/22）。5例HCL-V中有3例染色体核型异常。核糖体板层复合物（RLC）仅在约3/12的HCL病例中发现。在HCL-C中，包括嘌呤核苷类似物的化疗方案比其他方案达到了更好的完全缓解（CR）率（3/4 vs 1/18，P = 0.012）。中位随访期为27（1 - 142）个月。无失访情况。11例病情进展，6例死亡。中位总生存期（OS）未达到。1、3、6年OS率分别为84%、78%和58%。中位无进展生存期（PFS）为（63 ± 24）个月，1、2、5年PFS率分别为86%、72%和44%。HCL-V的中位PFS显著短于HCL-C（（23 ± 3）vs（78 ± 12）个月，P = 0.014）。在HCL-C组中，诊断时发热（P = 0.038）和贫血（P = 0.000）是不良预后因素。但嘌呤核苷类似物在PFS方面无显著差异。
中国HCL患者全血细胞减少不常见。电镜下经典RLC罕见。嘌呤核苷类似物可能达到更好的CR率，但未能提高PFS率。与HCL-C相比，HCL-V常见基因异常，预后较差，PFS较短。
