摘要:

AbstractAbstract Only transient engraftment of infused fetal liver cells has been demonstrated in a small proportion of patients with hypoplastic bone marrow or patients undergoing treatment for acute leukemia. This presumably reflects the ability of the recipient to reject the infused cells, the infusion of too few viable stem cells or the availability of too few accessory cells; it is clear from the successful engraftment of infused fetal liver cells in a high proportion of infants and fetuses with severe immunodeficiency diseases that, under favorable circumstances, cells derived from human fetal liver are capable of establishing effective grafts and making a substantial contribution to he‐matopoiesis comparable to that of transplanted cells derived from the liver of the fetal mouse, rat, rabbit or dog.Significant clinical and hematological improvements have been described following infusions of fetal liver cells without evidence of engraftment These improvements have been attributed to the ability of the infused cells to promote regeneration of autologous hematopoiesis and to inhibit the growth of tumor cells. These possibilities are worthy of evaluation in relation to the production of putative regulators of cellular proliferation in the liver. Meanwhile a suppressor of tumor growth is being used to purge bone marrow prior to autologous transplantation.The generation in vitro of cells which possess the properties of hematopoietic stem cells generated in the liver—from cells which can be maintained as permanent cell lines—would transform hematopoietic cell replacement therapy, and the possibility may not be too unrealistic to contem

ISSN:1066-5099    

DOI:10.1002/stem.5530110614

出版商:John Wiley&Sons, Ltd.    

年代:1993

数据来源: WILEY

摘要:

AbstractAbstract Hemophilia A is an X‐Iinked bleeding disorder caused by a quantitative or qualitative defect of coagulation factor VIII. Since factor VI11 has been cloned and several intragenic and linked DNA polymorphisms discovered, DNA analysis is an accepted and commonly used method for carrier testing in hemophilia A. Both a direct method using detection of mutation and an indirect method based on linkage between the disease and DNA polymorphism are used for this purpose.In this study, DNA samples of 110 hemophilia A patients from 99 families were screened for factor VIII gene mutation using Southern blot analysis; in seven families, mutations were detected. In 13 females from six families with identified mutation, the direct diagnosis of carriers was performed.Four intragenic (Bcll, Xbal, Bgll and Mspl in F8C locus) and two linked polymorphisms (TaqI in DXS52 locus and BglII in DXS15 locus) were studied in members of 47 hemophilia A families. BcIl‐Xbal‐BgII haplotypes were analyzed in 90 unrelated X chromosomes. Eighteen out of 31 females (58%) were heterozygous for at least one intragenic polymorphism, and 29 out of 31 females (94%) were heterozygous for at least one intra‐ or extragenic polymorphism tested. Carrier diagnosis was made in 15 out of 25 possible carriers (60%) based on intragenic and in an additional 3 out of 25 (12%) only on linked polym

ISSN:1066-5099    

DOI:10.1002/stem.5530110615

出版商:John Wiley&Sons, Ltd.    

年代:1993

数据来源: WILEY

ISSN:1066-5099    

DOI:10.1002/stem.5530110603

出版商:John Wiley&Sons, Ltd.    

年代:1993

数据来源: WILEY