Fetal-Like Erythropoiesis during Recovery from Transient Erythroblastopenia of Childhood (TEC)
Fetal-Like Erythropoiesis during Recovery from Transient Erythroblastopenia of Childhood (TEC)
复制标题
儿童期短暂性成红细胞减少症 (TEC) 恢复期间的胎儿样红细胞生成
作者：
M. Link;B. Alter
摘要：胎儿样红细胞生成常伴随骨髓应激状态，如Diamond-Blackfan综合征和再生障碍性贫血。相反，儿童期一过性成红细胞减少症患者的红细胞在诊断时缺乏胎儿特征。本报告描述了9例儿童短暂性成红细胞减少症，在恢复期观察到短暂的胎儿样红细胞生成。这些患者最初表现为贫血、网织红细胞减少、红细胞大小与年龄相符、胎儿血红蛋白和i抗原水平低。然而，在恢复期，红细胞表现出一种或多种胎儿特征。这些包括胎儿血红蛋白增加（5例患者中的3例），i抗原的存在（6例患者中的4例）和大红细胞症（9例患者中的7例）。这些胎儿特征持续超过2周后，网织红细胞计数恢复正常。在诊断后的一年内，红细胞不含胎儿特征。推测：这里报告的观察结果表明，“胎儿样”红细胞特征可能代表骨髓发育不全恢复期间的正常反应模式。如果这一假设是正确的，那么在从各种病因的骨髓发育不良（例如，溶血性贫血并发的发育不全危象）。“胎儿特征”的程度和持续时间的巨大变化可能反映了个体患者恢复期间增殖红细胞区室调节的差异。虽然恢复期“胎儿样红细胞生成”的意义尚不明确，但它可能为血红蛋白合成的调节提供线索。
Summary: Fetal-like erythropoiesis frequently accompanies marrow stress conditions such as Diamond-Blackfan syndrome and aplastic anemia. In contrast, patients with transient erythroblastopenia of childhood have erythrocytes which lack fetal characteristics at the time of diagnosis. This report describes nine children with transient erythroblastopenia of childhood in whom transient, fetal-like erythropoiesis was observed during the period of recovery. These patients initially presented with anemia, reticulocytopenia, erythrocytes of normal size for age, low levels of fetal hemoglobin, and i-antigen. During the recovery period, however, erythrocytes manifested one or more fetal characteristics. These included an increased fetal hemoglobin (in three of five patients), the presence of i-antigen (in four of six patients), and macrocytosis (in seven of nine patients). These fetal characteristics persisted more than 2 wk after the reticulocyte count returned to normal. Within one year from diagnosis, red blood cells contained no fetal characteristics.Speculation: The observations reported here suggest that "fetal-like" red blood cell characteristics may represent a normal response pattern during recovery from marrow hypoplasia. If this hypothesis is correct, similar fetal erythrocyte characteristics should be presentin red blood cells of patients recovering from marrow hypoplasia of various etiologies (e.g., hypoplastic crises complicating hemolytic anemias). The great variability in degree and duration o "fetal characteristics" may reflect differences in the regulation of the proliferating erythroid compartment during recovery among individual patients. Although the significance of "fetal-like erythropoiesis" during recovery remains to be defined, it may provideclues to the regulation of hemoglobin synthesis.