CAJ | 학술논문

目的探讨T幼淋巴细胞白血病(T-PLL)临床与实验室特点及生存情况.方法回顾性分析2006年1月至2012年10月就诊的11例T-PLL患者资料,并进行随访.结果 11例T-pLL患者男9例,女2例,中位发病年龄56(19～69)岁.除3例外,其他患者白细胞计数均增高.但高白细胞者比例(9％)显著低于英国患者(75％)(P=0.000).患者外周血淋巴细胞计数中位数为17.22(0.58～148.83)×109/L,11例患者中9例增高.浅表淋巴结肿大和脾大是常见体征.LDH中位数260(119～587) U/L,9例患者中5例高于正常.所有患者血清β2微球蛋白(β2-MG)水平(2.80～7.27 mg/L)均高于正常值.患者均表达胸腺后T细胞免疫标志,所有检测者CD2、CD3、CD5、TCRαβ均阳性,CD1a、TdT、HLA-DR均阴性,CD7多强阳性.仅1例患者为2号染色体单体,其他9例患者均为正常染色体核型,未见14qll异常和8q三体.而英国和美国患者正常核型比例分别为3％和25％,与我国患者比较差异均有统计学意义(P=0.000、P＝0.001).中位随访23.0(4～84)个月,3例死亡,2年无进展生存率为53.3％,2年总生存率为50.0％.其中3例患者无进展生存达数年,是否应考虑为T-慢性淋巴细胞白血病(T-CLL)值得进一步研究.结论 T-PLL临床表现多为淋巴细胞计数增高及浅表淋巴结肿大和脾大,患者血清LDH、β2-MG水平常升高,多数患者染色体核型正常.
목적 탐토T유림파세포백혈병(T-PLL)림상여실험실특점급생존정황.방법 회고성분석2006년1월지2012년10월취진적11례T-PLL환자자료,병진행수방.결과 11례T-pLL환자남9례,녀2례,중위발병년령56(19～69)세.제3예외,기타환자백세포계수균증고.단고백세포자비례(9％)현저저우영국환자(75％)(P=0.000).환자외주혈림파세포계수중위수위17.22(0.58～148.83)×109/L,11례환자중9례증고.천표림파결종대화비대시상견체정.LDH중위수260(119～587) U/L,9례환자중5례고우정상.소유환자혈청β2미구단백(β2-MG)수평(2.80～7.27 mg/L)균고우정상치.환자균표체흉선후T세포면역표지,소유검측자CD2、CD3、CD5、TCRαβ균양성,CD1a、TdT、HLA-DR균음성,CD7다강양성.부1례환자위2호염색체단체,기타9례환자균위정상염색체핵형,미견14qll이상화8q삼체.이영국화미국환자정상핵형비례분별위3％화25％,여아국환자비교차이균유통계학의의(P=0.000、P＝0.001).중위수방23.0(4～84)개월,3례사망,2년무진전생존솔위53.3％,2년총생존솔위50.0％.기중3례환자무진전생존체수년,시부응고필위T-만성림파세포백혈병(T-CLL)치득진일보연구.결론 T-PLL림상표현다위림파세포계수증고급천표림파결종대화비대,환자혈청LDH、β2-MG수평상승고,다수환자염색체핵형정상.
Objective To investigate the clinical and laboratory characteristics and survival of Chinese patients with T-cell prolymphocytic leukemia (T-PLL).Method Eleven patients with T-PLL admitted in our hospital from Jan 2006 to Oct 2012 were retrospectively analyzed.Results Of the 11 patients,nine were males and two females,with the median age of 56.0(19-69) years old.All the patients,except for three,presented with leukocytosis.The incidence of hyperleukocytosis (1/11) was less frequent than that in the British series (75％) (P =0.000).Lymphocyte counts in peripheral blood were increased in 9 of the 11 patients with the median absolute lymphocyte count (ALC) of 17.22 (0.58-148.83)× 109/L.Superficial lymphadenopathy and splenomegaly were the most common physical signs.It was common that serum lactate dehydrogenase (LDH) and beta 2 microglobulin (β2-MG) were higher than normal level.All cases were positive for CD2/CD3/CD5/TCRαβ,negative for CD1a/HLA-DR and TdT,and most of them were strong positive for CD7 expression.By chromosome analyses,most cases (9/10) have normal chromosome.This rate is significantly higher than that of the British and American series (3％ and 25％,respectively) (P =0.000,P =0.001).The 14qll abnormality and trisomy 8q,which are common among Western cases,were not observed in any of our cases.With a median follow-up of 23.0 months,three patients died.Two year progress free survival (PFS) and overall survival (OS) were 53.3％ and 50％,respectively.There were 3 patients with PFS over a number of years,whether it should be considered as the T-chronic lymphocytic leukemia (T-CLL) is worthy of further studies.Conclusion The common clinical manifestations of T-PLL patients were increased lymphocyte counts and lymphadenopathy as well as splenomegaly.And most cases have high level of blood LDH and β2-MG and normal chromosome karyotype.