Characterization of bone marrow stromal abnormalities in a patient with constitutional trisomy 8 mosaicism and myelodysplastic syndrome.

Abstract:

:The development of myeloid leukemias and myelodysplastic syndrome (MDS) is common in children with trisomy 8 mosaicism. However, the mechanisms by which the presence of an additional chromosome 8 translates to an increased risk of leukemias and MDS is currently unknown. The authors describe the analysis of stromal cells from a pediatric MDS patient with constitutional trisomy 8. Patient and control marrow stromal cells were analyzed for alterations in cytokine production. Clonogenic assays were used to examine stromal support for hematopoiesis. The interplay between leukemia cells and stroma was studied by co-culture experiments. The results indicate that stromal cell function in this patient was seriously altered in favor of progenitor cell proliferation and expansion. This indicates that constitutional trisomy 8 in stromal cells plays a critical role in the pathogenesis of MDS.

journal_name

Pediatr Hematol Oncol

authors

Narendran A,Hawkins LM,Ganjavi H,Vanek W,Gee MF,Barlow JW,Johnson G,Malkin D,Freedman MH

doi

10.1080/08880010490276917

subject

Has Abstract

pub_date

2004-04-01 00:00:00

pages

209-21

issue

3

eissn

0888-0018

issn

1521-0669

pii

RY7460CUH3038H9U

journal_volume

21

pub_type

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