中华血液学杂志
中華血液學雜誌
중화혈액학잡지
Chinese Journal of Hematology
2014年
8期
713-718
,共6页
覃霞%Irith Baumann%陈静%沈萍%陈洁枫%殷敏智
覃霞%Irith Baumann%陳靜%瀋萍%陳潔楓%慇敏智
담하%Irith Baumann%진정%침평%진길풍%은민지
贫血,难治性%骨髓增生异常综合征%贫血,再生障碍性%病理学,临床%免疫抑制治疗
貧血,難治性%骨髓增生異常綜閤徵%貧血,再生障礙性%病理學,臨床%免疫抑製治療
빈혈,난치성%골수증생이상종합정%빈혈,재생장애성%병이학,림상%면역억제치료
Anemia,refractory%Myelodysplastic syndrome%Anemia,aplastic%Pathology,clinical%Immunosuppressive therapy
目的 探讨儿童难治性血细胞减少(RCC)和获得性再生障碍性贫血(AAA)的临床特征和病理特征,为更好地诊断、鉴别诊断及治疗两种疾病患儿提供理论依据.方法 以130例RCC或AAA患儿为研究对象,回顾性分析其临床资料及骨髓活检病理特征.结果 130例患儿中78例(60.0％)为RCC,52例(40.0％)为AAA.RCC组患儿的外周血白细胞计数、中性粒细胞绝对值计数、血小板计数、血红蛋白、网织红细胞计数均显著高于AAA组(P＜0.01).RCC组患儿骨髓活检示增生低下,84.6％(66/78)的患儿骨髓造血细胞比例介于20％～ 60％,98.7％ (77/78)的患儿造血细胞呈典型的“不均匀片状”分布、红系造血细胞簇状分布伴核左移,61.5％(48/78)的患儿可见小巨核细胞.AAA组患儿骨髓活检示增生极为低下,88.5％(46/52)的患儿骨髓造血细胞比例＜5％,98.1％(51/52)的患儿骨髓中未见巨核细胞.给予环孢素±兔-抗胸腺细胞球蛋白±雄激素±中药的免疫抑制方案治疗,RCC和AAA患儿3个月总有效率分别为59.5％和26.9％ (P=0.011),6个月总有效率为75.0％和38.1％(P=0.007).结论 RCC患儿外周血细胞减少及骨髓增生减低程度较AAA轻,其骨髓造血细胞呈“不均匀片状”分布、红系造血细胞簇状分布伴核左移及小巨核细胞的出现为鉴别于AAA的典型病理学特征.RCC和AAA患儿接受免疫抑制方案治疗均可获得确切疗效,但RCC患儿的总有效率较AAA高.
目的 探討兒童難治性血細胞減少(RCC)和穫得性再生障礙性貧血(AAA)的臨床特徵和病理特徵,為更好地診斷、鑒彆診斷及治療兩種疾病患兒提供理論依據.方法 以130例RCC或AAA患兒為研究對象,迴顧性分析其臨床資料及骨髓活檢病理特徵.結果 130例患兒中78例(60.0％)為RCC,52例(40.0％)為AAA.RCC組患兒的外週血白細胞計數、中性粒細胞絕對值計數、血小闆計數、血紅蛋白、網織紅細胞計數均顯著高于AAA組(P＜0.01).RCC組患兒骨髓活檢示增生低下,84.6％(66/78)的患兒骨髓造血細胞比例介于20％～ 60％,98.7％ (77/78)的患兒造血細胞呈典型的“不均勻片狀”分佈、紅繫造血細胞簇狀分佈伴覈左移,61.5％(48/78)的患兒可見小巨覈細胞.AAA組患兒骨髓活檢示增生極為低下,88.5％(46/52)的患兒骨髓造血細胞比例＜5％,98.1％(51/52)的患兒骨髓中未見巨覈細胞.給予環孢素±兔-抗胸腺細胞毬蛋白±雄激素±中藥的免疫抑製方案治療,RCC和AAA患兒3箇月總有效率分彆為59.5％和26.9％ (P=0.011),6箇月總有效率為75.0％和38.1％(P=0.007).結論 RCC患兒外週血細胞減少及骨髓增生減低程度較AAA輕,其骨髓造血細胞呈“不均勻片狀”分佈、紅繫造血細胞簇狀分佈伴覈左移及小巨覈細胞的齣現為鑒彆于AAA的典型病理學特徵.RCC和AAA患兒接受免疫抑製方案治療均可穫得確切療效,但RCC患兒的總有效率較AAA高.
목적 탐토인동난치성혈세포감소(RCC)화획득성재생장애성빈혈(AAA)적림상특정화병리특정,위경호지진단、감별진단급치료량충질병환인제공이론의거.방법 이130례RCC혹AAA환인위연구대상,회고성분석기림상자료급골수활검병리특정.결과 130례환인중78례(60.0％)위RCC,52례(40.0％)위AAA.RCC조환인적외주혈백세포계수、중성립세포절대치계수、혈소판계수、혈홍단백、망직홍세포계수균현저고우AAA조(P＜0.01).RCC조환인골수활검시증생저하,84.6％(66/78)적환인골수조혈세포비례개우20％～ 60％,98.7％ (77/78)적환인조혈세포정전형적“불균균편상”분포、홍계조혈세포족상분포반핵좌이,61.5％(48/78)적환인가견소거핵세포.AAA조환인골수활검시증생겁위저하,88.5％(46/52)적환인골수조혈세포비례＜5％,98.1％(51/52)적환인골수중미견거핵세포.급여배포소±토-항흉선세포구단백±웅격소±중약적면역억제방안치료,RCC화AAA환인3개월총유효솔분별위59.5％화26.9％ (P=0.011),6개월총유효솔위75.0％화38.1％(P=0.007).결론 RCC환인외주혈세포감소급골수증생감저정도교AAA경,기골수조혈세포정“불균균편상”분포、홍계조혈세포족상분포반핵좌이급소거핵세포적출현위감별우AAA적전형병이학특정.RCC화AAA환인접수면역억제방안치료균가획득학절료효,단RCC환인적총유효솔교AAA고.
Objective To explore the clinical characteristics and histopathological morphology features of bone marrow biopsies between refractory cytopenia of children (RCC) and acquired aplastic anemia (AAA) to facilitate the diagnosis,differential diagnosis and treatment of RCC and AAA.Methods We retrospectively analyzed clinical data and histopathological morphology of bone marrow biopsies in RCC or AAA patients referred to our hospital from January 2011 to December 2012.Results There were totally 130 patients studied.The final diagnoses of them were RCC in 78 cases (60.0％) and AAA in 52 cases (40.0％).The median WBC count,absolute neutrophil count,blood platelet count,hemoglobin level,and reticulocyte count were all higher in RCC children than AAA (P＜0.01).All of RCC patients showed hypocellular biopsy specimens,and 84.6％ (66/78) of them had cellularity of bone marrow biopsy specimens ranging from 20％ to 60％.Patchy pattern distribution was seen in 98.7％ (77/78) of RCC cases,and micromegakaryocyte was found in 61.5％ (48/78) of RCC cases.All of AAA patients showed severe hypocellular biopsy specimens,and 88.5％ (46/52) of them had cellularity of bone marrow biopsy specimens under 5％.Megakaryocyte was not found in 98.1％ (51/52) of AAA cases.The response rates of immunosuppressive therapy using CsA ± rabbit anti-thymocyte globulin ± androgen ± traditional chinese medicine for patients with RCC and AAA were 59.5％ and 26.9％ at 3 months (P=0.011),and 75.0％ and 38.1％ at 6 months,respectively (P=0.007).Conclusion RCC patients showed milder cytopenia and bone marrow hyperplasia than AAA.Patchy distribution of hematopoietic cells,erythroid islands with a marked left shift and micromegakaryocytes were decisive histomorphological patterns used to separate RCC from SAA.Immunosuppressive therapy using CsA ± rabbit anti-thymocyte globulin ± androgen± traditional chinese medicine was an effective therapy in patients with RCC and AAA,and the outcome of immunosuppressive therapy for RCC patients was superior to that of AAA patients.
