CAJ | 학술논문

目的探讨我国毛细胞白血病(HCL)患者的临床表现与实验室特点、对治疗反应、生存及预后情况.方法回顾性总结1990年8月至2012年3月中国医学科学院北京协和医学院血液学研究所收治的30例HCL患者临床资料,并进行随访.结果经典型HCL患者22例(73.3％),变异型HCL患者8例(26.7％).脾大是HCL最常见的体征.变异型患者白细胞计数均增高,全血细胞减少在经典型患者占36.4％(8/22).5例行遗传学检查的变异型患者中3例染色体核型异常.12例患者骨髓电镜检查示3例核糖体-板层复合物阳性.在经典型患者,核苷类似物为主的化疗方案完全缓解(CR)率明显优于其他治疗方案(3/4比1/18,P=0.012).中位随访27(1 ～ 142)个月,无失访,11例进展,6例死亡.中位总生存(OS)时间未达到,预计1、3、6年OS率分别为84％、78％、58％.中位无进展生存时间(PFS)为(63±24)个月,预计1、2、5年PFS率分别为86％、72％、44％.变异型患者中位PFS明显短于经典型患者[(23±3)比(78±12)个月,P=0.014].单因素分析显示初诊发热、贫血为经典型患者预后不良因素(P=0.038、0.000),而核苷类似物使用与否不影响其疾病进展.结论我国HCL患者全血细胞减少者较低,电镜核糖体-板层复合物阳性率低.经典型患者使用核苷类似物可提高CR率,但不改善PFS.变异型HCL比例高于国外,常伴遗传学异常,且变异型患者PFS明显短于经典型,预后较差.
목적 탐토아국모세포백혈병(HCL)환자적림상표현여실험실특점、대치료반응、생존급예후정황.방법 회고성총결1990년8월지2012년3월중국의학과학원북경협화의학원혈액학연구소수치적30례HCL환자림상자료,병진행수방.결과 경전형HCL환자22례(73.3％),변이형HCL환자8례(26.7％).비대시HCL최상견적체정.변이형환자백세포계수균증고,전혈세포감소재경전형환자점36.4％(8/22).5례행유전학검사적변이형환자중3례염색체핵형이상.12례환자골수전경검사시3례핵당체-판층복합물양성.재경전형환자,핵감유사물위주적화료방안완전완해(CR)솔명현우우기타치료방안(3/4비1/18,P=0.012).중위수방27(1 ～ 142)개월,무실방,11례진전,6례사망.중위총생존(OS)시간미체도,예계1、3、6년OS솔분별위84％、78％、58％.중위무진전생존시간(PFS)위(63±24)개월,예계1、2、5년PFS솔분별위86％、72％、44％.변이형환자중위PFS명현단우경전형환자[(23±3)비(78±12)개월,P=0.014].단인소분석현시초진발열、빈혈위경전형환자예후불량인소(P=0.038、0.000),이핵감유사물사용여부불영향기질병진전.결론 아국HCL환자전혈세포감소자교저,전경핵당체-판층복합물양성솔저.경전형환자사용핵감유사물가제고CR솔,단불개선PFS.변이형HCL비례고우국외,상반유전학이상,차변이형환자PFS명현단우경전형,예후교차.
Objective To explore the clinical and laboratory characteristics and survival of Chinese patients with hairy cell leukemia (HCL).Methods A total of 30 HCL patients from August 1990 to March 2012 were retrospectively analyzed.Results 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=O.000) at diagnosis were poor prognostic factors.But purine nucleoside analogues made no significant difference in PFS.Conclusions 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.