Shi Xiao-Dong, Hu Tao, Feng Yan-Ling, Liu Rong, Li Jun-Hui, Wang Tian-You, Chen Jin
Department of Hematology, Children Hospital, Capital Institute of Pediatrics, Beijing 100020, China.
Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2004 Feb;12(1):74-8.
The study was aimed to observe morphological characteristics and hematopoiesis function of bone marrow megakaryocyte in children patients with myelodysplastic syndrome (MDS), and analyse the cause and mechanism of thrombocytopenia. CD41 McAb immunohistochemical technique was used to detect micromegakaryocytes of bone marrow smear. Plasma clot culture and CD41 McAb immunohistochemical technique were used for the MK-colony forming assay. The colony formations of CFU-MK and BFU-MK were measured. The results showed that there was no significant difference of CFU-MK colony formation rate between groups of MDS and control. But, in 62.5% of children patients the colony formation rate of CFU-MK decreased, in 25% increased, and in 12.5% was normal while BFU-MK formation rate decreased in MDS group significantly. The number of micromegakaryocyte and the positive rate of type I lymphoid micromegakaryocyte were significantly higher than those of the control group. In conclusion, there may be two kinds of megakaryocyte clones in bone marrow of children patients with MDS. One is supposed to be pathologic and potentially malignant micromegakaryocytes, the another may be the normal megakaryocytic precursors. The thrombocytopenia in MDS patients induced by increase of pathologic MK leads to abnormal development and maturation of MK in bone marrow.
本研究旨在观察小儿骨髓增生异常综合征（MDS）患者骨髓巨核细胞的形态学特征及造血功能，并分析血小板减少的原因及机制。采用CD41单克隆抗体免疫组化技术检测骨髓涂片微小巨核细胞。采用血浆凝块培养及CD41单克隆抗体免疫组化技术进行巨核细胞集落形成试验，检测CFU-MK和BFU-MK的集落形成情况。结果显示，MDS组与对照组CFU-MK集落形成率无显著差异，但MDS组62.5%患儿CFU-MK集落形成率降低，25%升高，12.5%正常，而BFU-MK形成率显著降低。微小巨核细胞数量及Ⅰ型淋巴样微小巨核细胞阳性率均显著高于对照组。结论：小儿MDS患者骨髓中可能存在两种巨核细胞克隆，一种为病理性且具有潜在恶性的微小巨核细胞，另一种可能为正常巨核细胞前体。MDS患者血小板减少是由病理性巨核细胞增多导致骨髓巨核细胞发育及成熟异常所致。
