摘要
目的研究获得性单纯无巨核细胞性血小板减少性紫癜(APATP)的发病机制。方法患者骨髓单个核细胞(MNC)以甲基纤维素培养法测定其CFUGM,CFUE和CFUMK。观察去除骨髓中T细胞和粘附细胞对CFUMK的影响。患者血清或IgG在培养前与正常或自身MNC孵育,检测对CFUMK的体液抑制作用。结果15例(53.6%)的发病由于CFUMK的内在缺陷。3例去T细胞,2例去单核巨噬细胞后,骨髓巨核细胞集落形成明显增加。6例(21.4%)血清抑制CFUMK,此种抑制物来自IgG,选择性地针对巨核细胞。2例发病机制未定。结论巨核祖细胞的内在缺陷是APATP的主要发病机制。部分患者可因异常免疫引起。MKCSA生成的失代偿可能也是一个重要病因。
Objective To investigate the possible pathogenesis of acquired pure amegakaryocytic thrombocytopenic purpura(APATP). Methods Twenty eight patients with APATP were studied. Bone marrow mononuclear cells(MNCs) from these patients were plated into methyl cellulose cultures for CFU GM, CFU E and CFU MK assay. The influence of depleting T cells or adherent cells from patients’ marrow cells on CFU MK growth was observed. The humoral inhibitory effect on CFU MK was determined by co incubation of patients’ sera or IgG with normal or autologous MNCs prior to cultures.The serum MK CSA was also assessed.Results Fifteen cases (53.6%) of APATP was resulted from intrinsic defect of CFU MK.The megakaryocyte colony formation was augmented significantly in 3 T lymphocytes depleted and 2 mono macrophages depleted patients. Sera from 6 patients(21.4%) were inhibitory to CFU MK. The inhibitor originated from IgG and selectively directed against the megakaryocyte. In the remaining 2 cases, the pathogenesis was not ascertained. Conclusion The intrinsic defect of megakaryocyte progenitor cell is considered to be a primary pathogenesis of APATP. In some patients the disease can result from abnormal immune mechanisms. The decompensation of MK CSA production could also be an important cause for APATP.
出处
《中华血液学杂志》
CAS
CSCD
北大核心
1999年第3期124-126,共3页
Chinese Journal of Hematology
关键词
紫癜
血小板减少性
巨核细胞
发病机制
Purpura, thrombocytopenic Megakaryocyte Hematopoietic stem cell Cell colony