Prindull G, Jentsch E, Hansmann I
Scand J Haematol. 1979 Jul;23(1):59-63. doi: 10.1111/j.1600-0609.1979.tb02855.x.
A 15-year-old boy with Fanconi's anaemia (FA) for 10 years developed acute erythroleukaemia. During the leukaemic phase, granulopoietic stem cells (CFUc) were absent from his bone marrow and blood, but proliferation and differentiation of bone marrow cells could be seen in semipermeable diffusion chambers in vivo, and globin synthesis of erythroblasts had become imbalanced. Chromosomal lesions of peripheral blood lymphocytes differed in the leukaemic phase from those in the pancytopenic phase. These data indicate that erythro-, myelo-, thrombo-, and lymphocytic cell lines all were involved in both the leukaemic and the pancytopenic process. It is suggested that terminal myeloproliferative disease developed as part of the natural history of FA.
一名患范科尼贫血（FA）10年的15岁男孩发生了急性红白血病。在白血病期，其骨髓和血液中缺乏粒细胞系干细胞（CFUc），但在体内半透性扩散小室中可见骨髓细胞的增殖和分化，且成红细胞的珠蛋白合成已变得失衡。外周血淋巴细胞的染色体病变在白血病期与全血细胞减少期有所不同。这些数据表明，红系、髓系、巨核系和淋巴细胞系均参与了白血病和全血细胞减少过程。提示终末期骨髓增殖性疾病是FA自然病程的一部分。
