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Fanconi贫血家族与白血病

The Family of Fanconi's Anemia and Leukemia

  • 摘要: 本文报道了2例Fanconi贫血(FA)兄弟,发病于10岁前。都患有全血细胞减少伴皮肤色素沉着,拇指缺如,生殖器发育不良等先天性畸形。经中西医治疗5年后,其中1例转变为急性白血病死亡,另1例一般健康状态良好。FA转变成白血病至今国内未见报道。本例家族的情形说明FA与原发性再障有质的不同。

     

    Abstract: Two siblings were suffered from Fanconi's anemia in their first decade.Their characteristic associated abnormalities include peripheral pancytopenia,hyperpigmentation,deformity of the thumbs and genital hypoplasia .One's disease transformed into leukemia in his 13 yrs old and the other's hemogram became very well by yrs treatment with traditional and western medicine.No previous report has been discoved of the association of Fanconi's anemia transforming leukemia in China .It indicated that the pathogenism of this family of Fanconi's anemia was differant from idiopathic aplastic anemia.

     

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