儿童期短暂性红细胞减少症。细胞介导的红细胞生成抑制的证据。

H Tamary, C Kaplinsky, S Shvartzmayer, T Umiel, M Pecht, S Levin, R Zaizov
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引用次数: 0

摘要

目的:描述了一名4岁半儿童短暂性红细胞减少症(TEC)的T细胞介导的红细胞发育不全。患者和方法:通过观察诊断时和恢复时骨髓红细胞祖细胞的集落生长情况来研究红细胞的生成情况。结果:诊断时全骨髓菌落形成单位-红细胞(CFU-E)生长仅为对照组的28%。患者骨髓t细胞耗竭后,CFU-E生长增加5倍以上,而对照骨髓中CFU-E抑制20%,BFU-E生长抑制40%。对照组和患者骨髓中集落形成单位-粒细胞-巨噬细胞(CFU-GM)的数量均未被所有这些操作显著改变。在恢复早期和晚期,CFU-E和BFU-E的生长明显改善,t细胞耗竭的作用减弱。外周血t抑制淋巴细胞数量增加,自然杀伤(NK)细胞活化,干扰素水平高,均与病毒感染一致。临床恢复与t抑制淋巴细胞数量的正常化有关。结论:结果表明,在这名TEC患儿中,先前的病毒感染可能引起了抑制性t细胞的激活和干扰素的分泌,从而导致细胞介导的红细胞生成抑制。
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Transient erythroblastopenia of childhood. Evidence for cell-mediated suppression of erythropoiesis.

Purpose: T cell-mediated red cell aplasia in a 4 1/2-year-old child with transient erythroblastopenia of childhood (TEC) is described.

Patients and methods: Erythropoiesis was studied by assessing the colony growth of marrow erythroid progenitors at the time of diagnosis and during recovery.

Results: The colony-forming unit-erythroid (CFU-E) growth of whole marrow at diagnosis was only 28% that of the control. T-cell depletion of the patient's marrow was followed by a more than fivefold increase in CFU-E growth, as compared with 20% inhibition of CFU-E and 40% inhibition of burst-forming unit-erythroid (BFU-E) growth in control marrow. The number of colony-forming unit-granulocyte-macrophage (CFU-GM) in both control and patient's marrow was not significantly altered by all of these manipulations. During early and late recovery, CFU-E and BFU-E growth improved substantially, and the effect of T-cell depletion diminished. Increased numbers of peripheral T-suppressor lymphocytes, as well as activation of natural killer (NK) cells and high levels of interferon, all consistent with viral infection, were found at presentation. Clinical recovery was associated with normalization of T-suppressor lymphocyte number.

Conclusions: The results suggest that in this child with TEC, a preceding viral infection may have caused activation of suppressor T-cells and interferon secretion leading to cell-mediated suppression of erythropoiesis.

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