|
|
 Previous Article | Table of Contents | Next Article 
Accelerated programmed cell death (apoptosis) in erythroid precursors of
patients with severe beta-thalassemia (Cooley's anemia) [see comments]
J Yuan, E Angelucci, G Lucarelli, M Aljurf, LM Snyder, CR Kiefer, L Ma and SL Schrier
Divisione Ematologica di Muraglia, Centro Trapianto di Midollo Osseo,
Ospedale Di Pesaro, Italy.
The profound and life-threatening anemia in patients with Cooley's anemia
is ascribed primarily to intramedullary hemolysis (ineffective
erythropoiesis), the cause of which is obscure. Based on prior morphologic
data showing nuclear abnormalities, we hypothesized that accelerated
apoptosis could occur in these erythroid precursors. The highly successful
bone marrow (BM) transplantation program for patients with Cooley's anemia
provided us with a unique opportunity to test this hypothesis. We obtained
pretransplantation BM aspiration samples from patients undergoing BM
transplantation in Pesaro, Italy and from their allogeneic donors. The
erythroid precursors were isolated using ficoll sedimentation and then
panning selecting fro CD45- cells. Cytospin and Giemsa staining showed that
the separation provided greater than 90% erythroblasts. Five million of
these erythroblasts were lysed and their DNA was isolated. There were
obvious ladder patterns of DNA breakdown products in beta-thalassemia major
samples, with less occurring in beta- thalassemia trait. Normal individuals
showed only a slight smear of breakdown of DNA. These results indicate
there is enhanced apoptosis in the erythroblasts in the BMs of Cooley's
anemia patients. This finding might partially explain why most of these
erythroblasts never survive to become mature erythrocytes.
Volume 82,
Issue 2,
pp. 374-377,
07/15/1993
Copyright © 1993 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:
|
|
|
|
 
I. V. Libani, E. C. Guy, L. Melchiori, R. Schiro, P. Ramos, L. Breda, T. Scholzen, A. Chadburn, Y. Liu, M. Kernbach, et al.
Decreased differentiation of erythroid cells exacerbates ineffective erythropoiesis in {beta}-thalassemia
Blood,
August 1, 2008;
112(3):
875 - 885.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
S. P. Perrine
Fetal Globin Induction--Can It Cure {beta} Thalassemia?
Hematology,
January 1, 2005;
2005(1):
38 - 44.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
F. Centis, L. Tabellini, G. Lucarelli, O. Buffi, P. Tonucci, B. Persini, M. Annibali, R. Emiliani, A. Iliescu, S. Rapa, et al.
The importance of erythroid expansion in determining the extent of apoptosis in erythroid precursors in patients with beta -thalassemia major
Blood,
November 15, 2000;
96(10):
3624 - 3629.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
P. Pootrakul, P. Sirankapracha, S. Hemsorach, W. Moungsub, R. Kumbunlue, A. Piangitjagum, P. Wasi, L. Ma, and S. L. Schrier
A correlation of erythrokinetics, ineffective erythropoiesis, and erythroid precursor apoptosis in Thai patients with thalassemia
Blood,
October 1, 2000;
96(7):
2606 - 2612.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
N. F. Olivieri
The {beta}-Thalassemias
N. Engl. J. Med.,
July 8, 1999;
341(2):
99 - 109.
[Full Text]
[PDF]
|
|
|
|
|
|
|
T. Ikuta, Y. W. Kan, P. S. Swerdlow, D. V. Faller, and S. P. Perrine
Alterations in Protein-DNA Interactions in the gamma -Globin Gene Promoter in Response to Butyrate Therapy
Blood,
October 15, 1998;
92(8):
2924 - 2933.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
F. A. Kuypers, J. Yuan, R. A. Lewis, L. M. Snyder, C. R. Kiefer, A. Bunyaratvej, S. Fucharoen, L. Ma, L. Styles, K. de Jong, et al.
Membrane Phospholipid Asymmetry in Human Thalassemia
Blood,
April 15, 1998;
91(8):
3044 - 3051.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
C. Rothmann, A. M. Cohen, and Z. Malik
Chromatin Condensation in Erythropoiesis Resolved by Multipixel Spectral Imaging: Differentiation Versus Apoptosis
J. Histochem. Cytochem.,
August 1, 1997;
45(8):
1097 - 1108.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
