中华血液学杂志
中華血液學雜誌
중화혈액학잡지
Chinese Journal of Hematology
2008年
5期
312-315
,共4页
周康%王慧君%张莉%李洪强%杨栋林%阎嶂松%井丽萍%吴玉红%储榆林%张凤奎
週康%王慧君%張莉%李洪彊%楊棟林%閻嶂鬆%井麗萍%吳玉紅%儲榆林%張鳳奎
주강%왕혜군%장리%리홍강%양동림%염장송%정려평%오옥홍%저유림%장봉규
白血病,淋巴细胞%贫血,再生障碍,纯红细胞
白血病,淋巴細胞%貧血,再生障礙,純紅細胞
백혈병,림파세포%빈혈,재생장애,순홍세포
Leukemia,lymphocytic%Anemia,aplastic,pure red cell
目的 提高对T细胞大颗粒淋巴细胞白血病(T-LGLL)合并纯红细胞再生障碍(PRCA)的认识.方法 回顾性分析我院2000年1月至2006年8月间确诊的T-LGLL合并PRCA患者临床及实验室检查特征.结果14例T-LGLL合并PRCA患者中男7例,女7例,中位年龄61岁.患者呈慢性病程,主要表现贫血症状,9例患者脾脏轻、中度肿大,1例患者同时伴轻度肝脏肿大,1例患者浅表淋巴结肿大.初诊时患者中位Hb 61.5 g/L,中位WBC 4.30(2.70～7.95)×109/L,中位淋巴细胞比例0.59(0.30～0.87),外周血大颗粒淋巴细胞中位比例和中位绝对值分别为0.36(0.14～0.77)和1.9(0.4～4.5)×109/L,骨髓有核细胞中大颗粒淋巴细胞中位比例0.165(0.085～0.410).部分患者血清学检查异常.12例患者常规染色体检查无异常.给予环孢素和(或)糖皮质激素为主的免疫抑制治疗,患者总有效率91%.结论 合并PRCA的T-LGLL患者临床及实验室特征与经典T-LGLL者相似,以贫血为突出表现,脾脏肿大多见.外周血白细胞正常或减少,大颗粒淋巴细胞相对增多.对免疫抑制治疗反应良好.
目的 提高對T細胞大顆粒淋巴細胞白血病(T-LGLL)閤併純紅細胞再生障礙(PRCA)的認識.方法 迴顧性分析我院2000年1月至2006年8月間確診的T-LGLL閤併PRCA患者臨床及實驗室檢查特徵.結果14例T-LGLL閤併PRCA患者中男7例,女7例,中位年齡61歲.患者呈慢性病程,主要錶現貧血癥狀,9例患者脾髒輕、中度腫大,1例患者同時伴輕度肝髒腫大,1例患者淺錶淋巴結腫大.初診時患者中位Hb 61.5 g/L,中位WBC 4.30(2.70～7.95)×109/L,中位淋巴細胞比例0.59(0.30～0.87),外週血大顆粒淋巴細胞中位比例和中位絕對值分彆為0.36(0.14～0.77)和1.9(0.4～4.5)×109/L,骨髓有覈細胞中大顆粒淋巴細胞中位比例0.165(0.085～0.410).部分患者血清學檢查異常.12例患者常規染色體檢查無異常.給予環孢素和(或)糖皮質激素為主的免疫抑製治療,患者總有效率91%.結論 閤併PRCA的T-LGLL患者臨床及實驗室特徵與經典T-LGLL者相似,以貧血為突齣錶現,脾髒腫大多見.外週血白細胞正常或減少,大顆粒淋巴細胞相對增多.對免疫抑製治療反應良好.
목적 제고대T세포대과립림파세포백혈병(T-LGLL)합병순홍세포재생장애(PRCA)적인식.방법 회고성분석아원2000년1월지2006년8월간학진적T-LGLL합병PRCA환자림상급실험실검사특정.결과14례T-LGLL합병PRCA환자중남7례,녀7례,중위년령61세.환자정만성병정,주요표현빈혈증상,9례환자비장경、중도종대,1례환자동시반경도간장종대,1례환자천표림파결종대.초진시환자중위Hb 61.5 g/L,중위WBC 4.30(2.70～7.95)×109/L,중위림파세포비례0.59(0.30～0.87),외주혈대과립림파세포중위비례화중위절대치분별위0.36(0.14～0.77)화1.9(0.4～4.5)×109/L,골수유핵세포중대과립림파세포중위비례0.165(0.085～0.410).부분환자혈청학검사이상.12례환자상규염색체검사무이상.급여배포소화(혹)당피질격소위주적면역억제치료,환자총유효솔91%.결론 합병PRCA적T-LGLL환자림상급실험실특정여경전T-LGLL자상사,이빈혈위돌출표현,비장종대다견.외주혈백세포정상혹감소,대과립림파세포상대증다.대면역억제치료반응량호.
Objective To analyze the characteristics of acquired pure red cell aplasia(PRCA) secondary to T cell large granular lymphocyte leukemia(T-LGLL). Methods Fourteen patients with T-LGLL associated with PRCA between 2000 and 2006 in our hospital were retrospectively analyzed. Results The median age at diagnosis was 61 years with equal gender distribution. The PRCA had indolent process, mainly presenting with anemia. Of the 14 patients, 9 had mild to moderate splenomegaly, one hepatomegaly and one lymphadenopathy. The median Hb level was 61.5 g/L and the median WBC count 4.3×109/L. The median percentage and count of LGL in peripheral blood were 0.36 and 1.9×109/L respectively. The median percentage of LGL in BM was 0. 165 (0. 085-0. 410). Some patients had serologic abnormalities. All the 12 cases with available bone marrow cell cytogenetics showed normal karyotypes. With cyclosporine A or glucocoticoid immunosuppressive therapy, the overall response was 91%. Conclusion T-LGLL was one of the major causes of acquired PRCA. This type of PRCA has the similar clinical and laboratory feature to that of other type of PRCA and has a good response to immunosuppressive therapy.
