摘要
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AA16例、RCC34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。
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 ane- mia cases from 2007-2010 in our hospital and collected clinical data. Experienced hematologists and patholo- gists evaluated bone marrow biopsy and smear respectively. Results Of 50 cases, 23 were male and 27 fe- male （M： F = 1：1.17 ） ,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 aplas- tic anemia（AA） and pathologists as RCC ,2 cases vice versa. The final diagnoses of 50 patients reached con- sensus between hematologists and pathologists were AA 16 cases ,RCC 34 cases including 8 refractory eytope- nias with muhilineage 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 con- fused with AA. The main points of differential were present dysplastic changes of megakaryocyte best appreci- ated by the hematologists and morphologists and abnormal location of hematopoietie 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.
出处
《中华血液学杂志》
CAS
CSCD
北大核心
2012年第12期1042-1045,共4页
Chinese Journal of Hematology
基金
卫生部属（管）医院2010-2012年度临床学科重点项目
