HERTWIGS ANEMIA - CHARACTERIZATION OF THE STEM-CELL DEFECT

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  • 1 January 1983
    • journal article
    • research article
    • Vol. 61  (4) , 765-769
Abstract
Mice homozygous for Hertwig''s anemia, an/an, have a mild macrocytic anemia and are refractory to the potentially curative action of exogenously administered erythropoietin (EP). Both red and white blood cell numbers are decreased. One possible explanation is that a precursor stem cell population is in short supply. This possibility was examined through comparisons of CFU-S [spleen colony forming unit], BFU-E [erythropietic burst forming cell] and CFU-E [erythroid colony forming unit] numbers in an/an and +/+ mouse bone marrow. The response of erythroid-committed stem cells to EP in vitro has also been determined. EP resistance apparently is due to diminished numbers rather than decreased EP sensitivity of responsive cells. In mice with Hertwig''s anemia, the relative numbers of both BFU-E and CFU-E are reduced. The erythroid-committed stem cells are more severely affected than their progenitors, the CFU-S. Cells apparently are lost during the progressive differentiation from stem cell to erythrocyte.

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