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dc.contributor.authorMarsh, Judith C W
dc.contributor.authorWill, A J
dc.contributor.authorHows, J M
dc.contributor.authorSartori, P
dc.contributor.authorDarbyshire, P J
dc.contributor.authorWilliamson, Peter J
dc.contributor.authorOscier, D G
dc.contributor.authorDexter, T Michael
dc.contributor.authorTesta, Nydia G
dc.date.accessioned2010-08-03T11:02:58Z
dc.date.available2010-08-03T11:02:58Z
dc.date.issued1992-06-15
dc.identifier.citation"Stem cell" origin of the hematopoietic defect in dyskeratosis congenita. 1992, 79 (12):3138-44 Blooden
dc.identifier.issn0006-4971
dc.identifier.pmid1596563
dc.identifier.urihttp://hdl.handle.net/10541/108921
dc.description.abstractWe have used the long-term bone marrow culture (LTBMC) system to analyze hematopoiesis in three patients with dyskeratosis congenita (DC), two of whom had aplastic anemia, and the third had a normal blood count (apart from mild macrocytosis) and normal BM cellularity. Hematopoiesis was severely defective in all three patients, as measured by a low incidence of colony-forming cells and a low level of hematopoiesis in LTBMC. The function of the marrow stroma was normal in its ability to support the growth of hematopoietic progenitors from normal marrows seeded onto them in all three cases, but the generation of hematopoietic progenitors from patients marrow cells inoculated onto normal stromas was reduced, thus suggesting the defect to be of stem cell origin. The parents and unaffected brother of one of the families have also been studied in LTBMC and all showed normal hematopoietic and stromal cell function. From this study we speculate that there are some similarities between DC and the defect in the W/Wv mouse.
dc.language.isoenen
dc.subjectHaematopoiesisen
dc.subjectHaematopoietic Stem Cellsen
dc.subject.meshAdolescent
dc.subject.meshAdult
dc.subject.meshBone Marrow
dc.subject.meshCells, Cultured
dc.subject.meshChild
dc.subject.meshErythrocyte Count
dc.subject.meshErythroid Precursor Cells
dc.subject.meshFemale
dc.subject.meshGranulocytes
dc.subject.meshHematopoiesis
dc.subject.meshHematopoietic Stem Cells
dc.subject.meshHumans
dc.subject.meshLeukocyte Count
dc.subject.meshLeukoplakia, Oral
dc.subject.meshMacrophages
dc.subject.meshMale
dc.subject.meshNails
dc.subject.meshPigmentation Disorders
dc.title"Stem cell" origin of the hematopoietic defect in dyskeratosis congenita.en
dc.typeArticleen
dc.contributor.departmentCRC Department of Experimental Hematology, Paterson Institute for Cancer Research, Christie Hospital, Manchester, UK.en
dc.identifier.journalBlooden
html.description.abstractWe have used the long-term bone marrow culture (LTBMC) system to analyze hematopoiesis in three patients with dyskeratosis congenita (DC), two of whom had aplastic anemia, and the third had a normal blood count (apart from mild macrocytosis) and normal BM cellularity. Hematopoiesis was severely defective in all three patients, as measured by a low incidence of colony-forming cells and a low level of hematopoiesis in LTBMC. The function of the marrow stroma was normal in its ability to support the growth of hematopoietic progenitors from normal marrows seeded onto them in all three cases, but the generation of hematopoietic progenitors from patients marrow cells inoculated onto normal stromas was reduced, thus suggesting the defect to be of stem cell origin. The parents and unaffected brother of one of the families have also been studied in LTBMC and all showed normal hematopoietic and stromal cell function. From this study we speculate that there are some similarities between DC and the defect in the W/Wv mouse.


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