Ritchey A K, Dainiak N, Hoffman R
Yale J Biol Med. 1985 Jan-Feb;58(1):1-8.
Transient erythroblastopenia of childhood (TEC) is a pure red cell aplasia which primarily affects children in the infant and toddler age group. The clinical syndrome of TEC is well defined and is characterized by moderate to severe anemia with reticulocytopenia, selective aplasia of the erythroid bone marrow elements, and spontaneous recovery, usually within a month of presentation. We utilized the plasma clot tissue culture technique to explore the defect of erythropoiesis in seven patients with TEC. Culture of bone marrow at diagnosis in four patients revealed an increased erythroid proliferative capacity in one and a decreased capacity in three. The former patient plus three additional patients were found to have a transient serum inhibitor of erythroid colony formation in autologous and allogeneic systems. The three patients with diminished erythroid proliferative capacity had no demonstrable serum inhibitor, and in one patient studied the erythroid proliferative capacity became supernormal after recovery. We conclude that although TEC has a characteristic clinical picture, in vitro studies reveal a variable expression of the erythropoietic defect and support the hypothesis of a heterogeneous pathogenesis of this disorder.
儿童 transient erythroblastopenia（TEC）是一种单纯红细胞再生障碍性贫血，主要影响婴幼儿年龄组的儿童。TEC 的临床综合征已明确界定，其特征为中度至重度贫血伴网织红细胞减少、红系骨髓成分选择性再生障碍以及通常在发病后一个月内自发恢复。我们利用血浆凝块组织培养技术探究了 7 例 TEC 患者的红细胞生成缺陷。4 例患者诊断时骨髓培养显示，1 例红系增殖能力增强，3 例能力降低。前 1 例患者加上另外 3 例患者在自体和异体系统中均发现有短暂的血清红系集落形成抑制剂。3 例红系增殖能力降低的患者未发现可证实的血清抑制剂，且在 1 例接受研究的患者恢复后红系增殖能力变得超常。我们得出结论，尽管 TEC 有特征性临床表现，但体外研究显示红细胞生成缺陷存在可变表达，并支持该疾病发病机制异质性的假说。
