|
|
 Previous Article | Table of Contents | Next Article 
Expression of embryonic globins by erythroid cells in juvenile chronic
myelocytic leukemia
T Papayannopoulou, B Nakamoto, NP Anagnou, D Chui, L Dow and J Sanders
Department of Medicine, University of Washington, Seattle 98195.
Juvenile chronic myelocytic leukemia (JCML) is a rare hematopoietic
neoplasia of early childhood with distinct hematologic and biochemical
features. We studied the biologic properties and the globin synthetic
profiles of JCML erythroid cells both in vivo and in vitro from a total of
24 patients. In these cases we observed the exuberant colony-forming
unit-macrophage (CFU-M) colony growth, as reported previously. Furthermore,
in contrast to previous reports, we found significant erythroid colony
growth in most of our cases (average: 1,182 burst- forming unit-erythroid
[BFUe] per 10(5) plated cells, range: 40 to 6,927). This growth was by and
large erythropoietin-dependent and was not greatly influenced by other
added cytokines. By several criteria all erythroid colony growth detected
in vitro was derived from JCML progenitors. The globin synthetic profile of
JCML erythroid cells showed high levels of fetal hemoglobin both in vivo
and in vitro (gamma/gamma + beta: 53% to 94% in reticulocytes, 62% to 98%
in BFUe- derived cells). In addition (in seven cases studied) we detected
embryonic globins (epsilon and zeta) at the protein and messenger RNA
level, a novel finding for primary leukemic cells. We speculate that the
transformed erythroid cells in JCML harbor a trans environment supporting
expression of developmentally earlier genes (fetal, embryonic). However, in
contrast to other acute or subacute leukemias, JCML erythroid cells also
have the ability to reach full maturation to the red cell level, thus
allowing detection of this primitive program in vivo.
Volume 77,
Issue 12,
pp. 2569-2576,
06/15/1991
Copyright © 1991 by The American Society of Hematology
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
B. S. Braun, J. A. Archard, J. A. G. Van Ziffle, D. A. Tuveson, T. E. Jacks, and K. Shannon
Somatic activation of a conditional KrasG12D allele causes ineffective erythropoiesis in vivo
Blood,
September 15, 2006;
108(6):
2041 - 2044.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. Schubbert, K. Lieuw, S. L. Rowe, C. M. Lee, X. Li, M. L. Loh, D. W. Clapp, and K. M. Shannon
Functional analysis of leukemia-associated PTPN11 mutations in primary hematopoietic cells
Blood,
July 1, 2005;
106(1):
311 - 317.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
D. P. Steensma, R. J. Gibbons, and D. R. Higgs
Acquired {alpha}-thalassemia in association with myelodysplastic syndrome and other hematologic malignancies
Blood,
January 15, 2005;
105(2):
443 - 452.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
L. J. N. Cooper, K. M. Shannon, M. R. Loken, M. Weaver, K. Stephens, and E. L. Sievers
Evidence that juvenile myelomonocytic leukemia can arise from a pluripotential stem cell
Blood,
September 15, 2000;
96(6):
2310 - 2313.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
H.Y. Luo, X.L. Liang, C. Frye, M. Wonio, G.D.V. Hankins, D.H.K. Chui, and B.P. Alter
Embryonic Hemoglobins Are Expressed in Definitive Cells
Blood,
July 1, 1999;
94(1):
359 - 361.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. Arico, A. Biondi, and C.-H. Pui
Juvenile Myelomonocytic Leukemia
Blood,
July 15, 1997;
90(2):
479 - 488.
[Full Text]
[PDF]
|
|
|
|
|
