de Alarcon P A, Mazur E M, Schmieder J A
Department of Pediatrics, University of Iowa, Iowa City 52242.
Am J Pediatr Hematol Oncol. 1987 Fall;9(3):212-8.
Idiopathic thrombocytopenic purpura (ITP) of childhood is a disorder characterized by a history of previous viral illness followed by acute onset of low circulating platelet count with present or increased megakaryocytes in the bone marrow. The majority of children recover a normal platelet count within 6 months to 1 year after onset of the disease. To better understand the regulation of megakaryocytopoiesis in this disorder, we studied nine patients with childhood ITP with the plasma clot colony assay in vitro for megakaryocyte colony forming units (CFU-Mk). Mononuclear bone marrow cells from some of the patients with ITP contained greater numbers of CFU-Mk and greater numbers of cells per colony than mononuclear bone marrow cells from healthy adult volunteers (p less than 0.026) when the cultures contained no added megakaryocyte colony-stimulating activity (Mk-CSA). The serum from patients with ITP did not stimulate in vitro megakaryocytopoiesis from healthy adult volunteers' bone marrow mononuclear cells above baseline values. These findings are consistent with the hypothesis that a decrease in bone marrow megakaryocytes is needed for Mk-CSA production. Alternatively, Mk-CSA is consumed by active megakaryocytopoiesis in the bone marrow.
儿童特发性血小板减少性紫癜(ITP)是一种具有以下特征的病症:既往有病毒感染病史,随后急性出现循环血小板计数降低,骨髓中存在或增加巨核细胞。大多数儿童在疾病发作后6个月至1年内血小板计数恢复正常。为了更好地理解这种病症中巨核细胞生成的调节机制,我们对9例儿童ITP患者进行了研究,采用体外血浆凝块集落试验检测巨核细胞集落形成单位(CFU-Mk)。当培养物中未添加巨核细胞集落刺激活性(Mk-CSA)时,部分ITP患者的骨髓单个核细胞所含CFU-Mk数量以及每个集落中的细胞数量均多于健康成年志愿者的骨髓单个核细胞(p<0.026)。ITP患者的血清并未刺激健康成年志愿者骨髓单个核细胞的体外巨核细胞生成超过基线值。这些发现与以下假设一致:Mk-CSA产生需要骨髓巨核细胞减少。或者,Mk-CSA被骨髓中活跃的巨核细胞生成所消耗。