RED-CELL HYPOPLASIA AND INCREASED BONE-MARROW RETICULIN IN SYSTEMIC LUPUS ERYTHEMATOSIS - REVERSAL WITH CORTICOSTEROID-THERAPY
RED-CELL HYPOPLASIA AND INCREASED BONE-MARROW RETICULIN IN SYSTEMIC LUPUS ERYTHEMATOSIS - REVERSAL WITH CORTICOSTEROID-THERAPY
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DOI：
10.1002/ajh.2830050310
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发表时间：
1978-01-01
影响因子：
12.8
通讯作者：
MENDELOW, H
中科院分区：
文献类型：
--
作者：
CAVALCANT, J;SHADDUCK, RK;MENDELOW, H
一名29岁中国男性患上严重再生障碍性贫血。骨髓弥漫性细胞增多，骨髓网硬蛋白增加，红系分化持续失败，超过原成红细胞阶段。虽然他没有表现出典型的系统性红斑狼疮的特征，但多项血清学研究与此诊断雅阁。通过体外红系细胞集落生长技术研究了患者的红细胞生成缺陷。尽管有严重的红细胞发育不全，患者的骨髓产生了丰富的大红细胞集落。血清促红细胞生成素活性高，通过使用该体外测定法判断。虽然患者的天然血清不影响集落形成，但分离的IG[免疫球蛋白]G组分对集落生长具有显著抑制作用。红细胞发育不全可能是由一种独特的自身抗体引起的。患者的血液学异常在皮质类固醇治疗后完全逆转。
A 29 yr old Chinese male developed severe aregenerative anemia. The bone marrow was diffusely hypercellular with increased marrow reticulin and a persistent failure of erythroid differentiation beyond the pronormoblast stage. Although he did not manifest classic features of systemic lupus erythematosus, multiple serologic studies were in accord with this diagnosis. The patient''s defect in erythropoiesis was studied by an in vitro technique for the growth of erythroid colonies. Despite the severe erythroid hypoplasia, the patient''s marrow yielded abundant large erythroid colonies. Serum erythropoietin activity was high as judged by use of this in vitro assay. Although the patient''s native serum did not affect colony formation, a separated Ig[immunoglobulin]G fraction was markedly inhibitory to colony growth. The erythroid hypoplasia may have resulted from a unique autoantibody. The patient''s hematologic abnormalities completely reversed following treatment with corticosteroids.