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[先天性再生障碍性贫血(戴蒙德-布莱克范综合征)发病机制的研究]

[Studies on the pathogenesis of congenital hypoplastic anemia (Diamond-Blackfan syndrome)].

作者信息

Sawada K

出版信息

Hokkaido Igaku Zasshi. 1986 Jan;61(1):72-82.

PMID:3486151
Abstract

CFU-E growth from fractionated bone marrow cells of five patients with Diamond Blackfan syndrome was studied. In all patients, CFU-E growth was reduced in mononuclear cell rich fraction. In two patients out of five, CFU-E growth was returned to normal by the depletion of E-rosette forming cells of monocytes from mononuclear cell rich fraction. In the patient whose CFU-E growth returned to normal by the depletion of E-rosette forming cells, cocultivation between bone marrow buffy coat cells and autologous bone marrow E-rosette forming cells resulted in a significant decrease of CFU-E growth, and there was a significant increase in CFU-E growth by the treatment of OKT 4. In other three patients out of five, there was no significant increase in CFU-E growth by the depletion of monocytes and/or E-rosette forming cells. It is concluded that immunologic causes such as cellular factors may play a role, at least in part, on the pathogenesis of Diamond-Blackfan syndrome.

摘要

对五名先天性纯红细胞再生障碍性贫血综合征患者的分级骨髓细胞的集落形成单位-红细胞(CFU-E)生长情况进行了研究。在所有患者中,富含单核细胞的部分中CFU-E生长均降低。五名患者中有两名患者,通过从富含单核细胞的部分中去除单核细胞的E玫瑰花结形成细胞,CFU-E生长恢复正常。在通过去除E玫瑰花结形成细胞使CFU-E生长恢复正常的患者中,骨髓血沉棕黄层细胞与自体骨髓E玫瑰花结形成细胞之间的共培养导致CFU-E生长显著降低,并且通过OKT 4治疗CFU-E生长显著增加。在另外三名患者中,去除单核细胞和/或E玫瑰花结形成细胞后CFU-E生长没有显著增加。得出的结论是,诸如细胞因子等免疫因素可能至少部分地在先天性纯红细胞再生障碍性贫血综合征的发病机制中起作用。

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1
[Studies on the pathogenesis of congenital hypoplastic anemia (Diamond-Blackfan syndrome)].[先天性再生障碍性贫血(戴蒙德-布莱克范综合征)发病机制的研究]
Hokkaido Igaku Zasshi. 1986 Jan;61(1):72-82.
2
Diamond-Blackfan syndrome: a possible role of cellular factors for erythropoietic suppression.
Scand J Haematol. 1985 Aug;35(2):158-65. doi: 10.1111/j.1600-0609.1985.tb01565.x.
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Erythroid precursors in congenital hypoplastic (Diamond-Blackfan) anemia.先天性再生障碍性贫血(戴蒙德-布莱克范贫血)中的红系前体细胞。
J Clin Invest. 1978 Feb;61(2):489-98. doi: 10.1172/JCI108960.
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[A possible role of T cell subsets causing anemia in systemic lupus erythematosus (SLE)].[T细胞亚群在系统性红斑狼疮(SLE)所致贫血中可能发挥的作用]
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[Role of Th1 cell in the pathogenesis of aplastic anemia].[辅助性T细胞1在再生障碍性贫血发病机制中的作用]
Zhonghua Xue Ye Xue Za Zhi. 2002 Nov;23(11):574-7.
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In vitro bone marrow GM-CFC growth in aplastic patients. Correlation between E rosette forming cells and immunotherapy results.再生障碍性贫血患者体外骨髓粒-巨噬细胞集落形成细胞的生长。E玫瑰花结形成细胞与免疫治疗结果之间的相关性。
Nouv Rev Fr Hematol (1978). 1984;26(1):13-8.