中华血液学杂志
中華血液學雜誌
중화혈액학잡지
Chinese Journal of Hematology
2012年
12期
1042-1045
,共4页
李占琦%竺晓凡%杨文钰%刘恩彬%孙琦%方立环%孙福军%杨晴英%张培红
李佔琦%竺曉凡%楊文鈺%劉恩彬%孫琦%方立環%孫福軍%楊晴英%張培紅
리점기%축효범%양문옥%류은빈%손기%방립배%손복군%양청영%장배홍
儿童%病理学,临床%全血细胞减少%贫血,再生障碍性%诊断,鉴别
兒童%病理學,臨床%全血細胞減少%貧血,再生障礙性%診斷,鑒彆
인동%병이학,림상%전혈세포감소%빈혈,재생장애성%진단,감별
Child%Pathology,clincal%Pancytopenia%Anemia,aplastic%Diagnosis,differential
目的 探讨儿童难治性血细胞减少(refractory cytopenia of children,RCC)的诊断及鉴别诊断,并评价骨髓穿刺和活检在RCC诊断中的作用.方法 回顾性分析2007至2010年间50例临床诊断为非重型再生障碍性贫血(AA)患儿的临床资料,复查骨髓穿刺涂片、骨髓活检、血常规及免疫组化等实验室检查,根据骨髓增生异常综合征WHO分类标准(2008版)重新评估儿童全血细胞减少中RCC的诊断及相关的鉴别诊断.结果 50例患儿中男23例、女27例(男∶女=1∶1.17),诊断时中位年龄9(3~14)岁.骨髓穿刺涂片与骨髓活检诊断不一致的共有5例,其中3例涂片诊断为AA,活检诊断为RCC,2例反之.结合骨髓穿刺涂片和活检结果,最终诊断AA 16例、RCC 34例[其中8例为难治性血细胞减少伴多系发育异常(RCMD)].全部16例AA患者骨髓增生极度低下,RCC中有4例(15.38%)、RCMD有5例(62.50%)骨髓增生极度低下,RCC中有19例(73.08%)、RCMD中有2例(25.00%)骨髓增生较低下,RCC中有3例(11.54%)、RCMD有1例(12.50%)骨髓增生正常.结论 RCC骨髓原始细胞数并不增多,病态造血是主要特征.RCC与AA鉴别诊断要点:有否病态造血及程度、造血细胞的异常分布及定位、巨核细胞的形态特点等.AA与RCC患者鉴别诊断须骨髓穿刺涂片和活检两种检查手段互为补充.
目的 探討兒童難治性血細胞減少(refractory cytopenia of children,RCC)的診斷及鑒彆診斷,併評價骨髓穿刺和活檢在RCC診斷中的作用.方法 迴顧性分析2007至2010年間50例臨床診斷為非重型再生障礙性貧血(AA)患兒的臨床資料,複查骨髓穿刺塗片、骨髓活檢、血常規及免疫組化等實驗室檢查,根據骨髓增生異常綜閤徵WHO分類標準(2008版)重新評估兒童全血細胞減少中RCC的診斷及相關的鑒彆診斷.結果 50例患兒中男23例、女27例(男∶女=1∶1.17),診斷時中位年齡9(3~14)歲.骨髓穿刺塗片與骨髓活檢診斷不一緻的共有5例,其中3例塗片診斷為AA,活檢診斷為RCC,2例反之.結閤骨髓穿刺塗片和活檢結果,最終診斷AA 16例、RCC 34例[其中8例為難治性血細胞減少伴多繫髮育異常(RCMD)].全部16例AA患者骨髓增生極度低下,RCC中有4例(15.38%)、RCMD有5例(62.50%)骨髓增生極度低下,RCC中有19例(73.08%)、RCMD中有2例(25.00%)骨髓增生較低下,RCC中有3例(11.54%)、RCMD有1例(12.50%)骨髓增生正常.結論 RCC骨髓原始細胞數併不增多,病態造血是主要特徵.RCC與AA鑒彆診斷要點:有否病態造血及程度、造血細胞的異常分佈及定位、巨覈細胞的形態特點等.AA與RCC患者鑒彆診斷鬚骨髓穿刺塗片和活檢兩種檢查手段互為補充.
목적 탐토인동난치성혈세포감소(refractory cytopenia of children,RCC)적진단급감별진단,병평개골수천자화활검재RCC진단중적작용.방법 회고성분석2007지2010년간50례림상진단위비중형재생장애성빈혈(AA)환인적림상자료,복사골수천자도편、골수활검、혈상규급면역조화등실험실검사,근거골수증생이상종합정WHO분류표준(2008판)중신평고인동전혈세포감소중RCC적진단급상관적감별진단.결과 50례환인중남23례、녀27례(남∶녀=1∶1.17),진단시중위년령9(3~14)세.골수천자도편여골수활검진단불일치적공유5례,기중3례도편진단위AA,활검진단위RCC,2례반지.결합골수천자도편화활검결과,최종진단AA 16례、RCC 34례[기중8례위난치성혈세포감소반다계발육이상(RCMD)].전부16례AA환자골수증생겁도저하,RCC중유4례(15.38%)、RCMD유5례(62.50%)골수증생겁도저하,RCC중유19례(73.08%)、RCMD중유2례(25.00%)골수증생교저하,RCC중유3례(11.54%)、RCMD유1례(12.50%)골수증생정상.결론 RCC골수원시세포수병불증다,병태조혈시주요특정.RCC여AA감별진단요점:유부병태조혈급정도、조혈세포적이상분포급정위、거핵세포적형태특점등.AA여RCC환자감별진단수골수천자도편화활검량충검사수단호위보충.
Objective To explore the diagnosis and differential diagnosis of refractory cytopenia of children(RCC) according to WHO classification,and discuss the relationship between the cytology reviewed by hematologists and histology reviewed by pathologists. Methods We selected 50 non-severe aplastic anemia cases from 2007-2010 in our hospital and collected clinical data.Experienced hematologists and pathologists evaluated bone marrow biopsy and smear respectively. Results Of 50 cases, 23 were male and 27 female (M[JP16]∶[JP]F=1[JP16]∶[JP]1.17),the median age at diagnosis was 9 years(ranged from 3 to 14 years).5 patients had disagreement of diagnosis between hematologists and pathologists.In 3 cases hematologists diagnosed as aplastic anemia(AA）and pathologists as RCC,2 cases vice versa.The final diagnoses of 50 patients reached consensus between hematologists and pathologists were AA 16 cases,RCC 34 cases including 8 refractory cytopenias with multilineage dysplasia(RCMD) cases.All 16 cases AA showed severe hypocellularity.Only 4 cases(25.00%) RCC showed severe hypocellularity,19 cases(73.08%) RCC showed mild hypocellularity and 3 cases(11.54%) RCC were nomal hypocellularity. Conclusion Our results suggests that RCC was not rare in China. The main feature of RCC was dysplasia because of absence of increased blast. RCC was easily confused with AA. The main points of differential were present dysplastic changes of megakaryocyte best appreciated by the hematologists and morphologists and abnormal location of hematopoietic easily observed by pathologists. Overall, cytology and histology were complementary in the investigation of RCC and AA, because of sometimes one might give information that not be gived from the other.
