Greenberg B R, Wilson F D, Woo L, Knox S, Jenks H, Taplett J
Br J Haematol. 1981 May;48(1):85-93. doi: 10.1111/j.1365-2141.1981.00085.x.
To determine if abnormalities exist in the bone marrow stroma in Fanconi's anaemia, we studied the cytogenetic composition of in vitro bone marrow fibroblastic cells (FC) from a patient with this disorder and compared it to those obtained from skin fibroblasts, peripheral blood lymphocytes, and direct bone marrow preparations. The presence of granulocytic progenitors in bone marrow and T lymphocyte colonies in peripheral blood was also determined in addition to the ability of this patient's FC to stimulate normal granulocytic progenitors. We found that the FC had far fewer chromosomal abnormalities and stimulated normally the growth of granulocyte colonies. Granulocyte progenitors were not found, but T lymphocyte colonies were abundant. These results support the concept that a defect in haematopoietic stromal elements is not responsible for the aplasia developing in the disorder.
为了确定范可尼贫血患者的骨髓基质是否存在异常，我们研究了一名该疾病患者体外骨髓成纤维细胞（FC）的细胞遗传学组成，并将其与从皮肤成纤维细胞、外周血淋巴细胞和直接骨髓标本中获得的细胞遗传学组成进行了比较。除了该患者的FC刺激正常粒细胞祖细胞的能力外，还测定了骨髓中粒细胞祖细胞和外周血中T淋巴细胞集落的存在情况。我们发现FC的染色体异常要少得多，并且能正常刺激粒细胞集落的生长。未发现粒细胞祖细胞，但T淋巴细胞集落丰富。这些结果支持了造血基质成分缺陷不是该疾病中发育不全的原因这一观点。
