Aplastic anemia and stem cell biology:
Gespeichert in:
Format: | Buch |
---|---|
Sprache: | English |
Veröffentlicht: |
Philadelphia [u.a.]
Saunders
1997
|
Schriftenreihe: | Hematology, oncology clinics of North America
11,6 |
Schlagworte: | |
Online-Zugang: | Inhaltsverzeichnis |
Beschreibung: | XIV S., S. 1025 - 1259 Ill., graph. Darst. |
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490 | 1 | |a Hematology, oncology clinics of North America |v 11,6 | |
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650 | 4 | |a Anemia, Aplastic |x etiology | |
650 | 4 | |a Aplastic anemia |x Etiology | |
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Datensatz im Suchindex
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adam_text | I APLASTIC ANEMIA AND STEM CELL BIOLOGY
CONTENTS
Foreword: The Hematopoietic Stem Cell:
Present and Future xi
Stuart H. Orkin
Preface xiii
Leonard I. Zon
Clinical Aspects of Aplastic Anemia 1025
Eva C. Guinan
Severe aplastic anemia is a disorder characterized by peripheral
pancytopenia and marrow hypoplasia. Although its pathophysi
ology is understood poorly, the majority of patients appear to
have some immunologic destruction or suppression of hemato¬
poietic cells. The only curative therapy to date is allogeneic stem
cell transplantation, although the success of palliative immuno
suppressive therapies has improved over the last two decades.
Making the best therapy choice is complex and often requires
balancing very divergent toxicity profiles, both acute and long
term.
Molecular Biology of Fanconi Anemia 1045
Gary M. Kupfer, Dieter Naf, and Alan D. D Andrea
Fanconi anemia (FA) is a rare, autosomal recessive disease charac¬
terized by multiple congenital abnormalities, bone marrow fail¬
ure, and cancer susceptibility. Although traditionally described
as a classic clinical syndrome, as more is discovered regarding
its basic molecular and cell biology, FA is emerging as a true
premalignant syndrome. Two of the genes of the five known
HEMATOLOGY/ONCOLOGY CLINICS OF NORTH AMERICA
VOLUME 11 • NUMBER 6 • DECEMBER 1997 V
complementation groups have been cloned, and work to under¬
stand their function is underway. Further understanding of these
gene products has lent new ideas concerning modes of novel
therapy, including gene therapy. The impact of molecular biology
on our understanding of basic biology and the clinical care of FA
patients is discussed.
Diamond Blackfan Anemia 1061
Oleg I. Krijanovski and Colin A. Sieff
Diamond Blackfan anemia (DBA) is a rare, congenital, hypoplas
tic anemia that usually presents in early infancy. Congenital
anomalies, particularly of the head and upper limbs, are present
in about a quarter of reported patients. The disease is character¬
ized by a moderate to severe macrocytic anemia, occasional neu
tropenia or thrombocytosis, a normocellular bone marrow with
erythroid hypoplasia, and an increased risk of developing leuke¬
mia. The pathogenesis is unknown. The majority of patients re¬
spond to prednisone, and often erythropoiesis can be maintained
with low doses of the drug. Both remissions and increased resis¬
tance to steroid treatment can occur. Nonresponders usually are
transfusion dependent, although responses to high dose steroid,
androgen, and interleukin 3 have been observed. Bone marrow
transplantation can be curative.
Stem Cells in Chronic Myelogenous Leukemia 1079
Catherine M. Verfaillie
This article discusses briefly the molecular consequences of the
BCR ABL fusion gene. It then reviews the current evidence sup¬
porting the notion that chronic myelogenous leukemia in its
chronic phase is a clonal, hematopoietic, stem cell disease in
which malignant hematopoietic stem and progenitor cells re¬
spond to normal external proliferation and differentiation stim¬
uli, but in which such responses are altered owing to defects
in the stem and progenitor cells as a result of the BCR ABL
oncogene.
Developmental Biology of Hematopoiesis 1115
Todd Evans
Hematopoietic stem cells are at the top of a hierarchy that regu¬
lates the generation of a vast repertoire of blood cells during the
lifetime of a vertebrate. Recent experiments, using a vast variety
of embryonic systems, shed new light on the origin of stem cells
and the genes that function to regulate and maintain hematopoi¬
etic differentiation programs. Two distinct populations of stem
cells develop—derived initially from transient, extraembryonic
source and later from a stable, intraembryonic source; it is possi¬
ble that both are generated from a pro HSC able to respond
differentially to local inductions. The initial blood cells develop
Vi CONTENTS
from ventral mesoderm. The blood forming region develops as a
result of signaling from specific, secreted, embryonic growth fac¬
tors, including the bone morphogenetic proteins. Stem cells give
rise to progenitors that are restricted progressively in their ability
to contribute to specific lineages. This process is regulated by
transcription factors, whose functions are confirmed through ge¬
netic analyses. The identification of highly conserved, embryonic
signaling pathways and transcription regulatory genes illustrates
the enormous utility of analyzing embryonic hematopoiesis in
frog, chick, fish, and mouse systems to further our understanding
of human stem cells.
Intraembryonic Hematopoietic Stem Cells 1149
Franchise Dieterlen Lievre
Intraembryonic hematopoietic stem cells (HSC) were first de¬
tected in avian chimeras associating an embryo with a yolk sac
(YS). Cell markers were used to construct chimeras. The results
showed that YS blood precursors undergo primitive erythropoie
sis and become extinct, whereas intraembryonic precursors colo¬
nize rudiments of blood forming organs and settle in the bone
marrow as self renewable HSC. The model is valid in the mouse
as shown by in vitro cultures of cells obtained from embryo
structures or YS separated prior to circulation. This approach, as
well as restoration of irradiated adults, demonstrates that YS
precursors have a limited potential compared with embryo pre¬
cursors. The emergence of hematopoietic precursors in both YS
and embryos is closely linked to the emergence of the endothelial
network and is restricted to the mesoderm layer associated with
endoderm.
Growth Factors and Hematopoietic Stem Cells 1173
Ole P. Veiby, Adel A. Mikhail, and H. Ralph Snodgrass
The hematopoietic system is mediated in part by cell to cell inter¬
actions and soluble mediators or growth factors (cytokines). A
large number of cytokines directly and potently control hemato¬
poietic stem and precursor cell proliferation and differentiation.
This review focuses on the recent studies devoted to the role of
cytokines in the ex vivo expansion and differentiation of hemato¬
poietic stem and precursor cells.
Embryonic Stem Cells and Hematopoietic Stem
Cell Biology 1185
Mitchell J. Weiss
Two advances in murine embryonic stem (ES) cell technology
and their applications for the study of hematopoietic stem cells
(HSCs) are discussed in this article. First, ES cells induced to
differentiate in vitro form hematopoietic lineages in a fashion
that recapitulates the ontogeny of blood formation in the embryo.
CONTENTS Vii
This system offers a unique opportunity to isolate, examine, and
manipulate the most primitive hematopoietic progenitors. Sec¬
ond, targeted gene ablation (knockout) studies in ES cells have
identified several genes that are required for normal hematopoie
sis and may function in the formation, maintenance, and differen¬
tiation of HSCs. Insights into murine hematopoiesis gained
through the study of ES cells generally should be applicable to
other vertebrates, including humans.
Stem Cell Transcription Factors 1199
Ramesh A. Shivdasani
The individual properties of hematopoietic stem cells, including
self renewal, maintenance of pluripotency, and asymmetric cell
division, must depend at some level on the functions of specific
transcription factors. Recently, valuable insights into stem cell
transcription factor function have emerged from targeted gene
disruption (knockout) studies in mice. Absence of transcription
factors belonging to diverse protein families results in interfer¬
ence with expansion and differentiation of either the stem cell
itself or of primitive multipotential progenitors. The findings from
these and complementary experiments provide a framework for
examining the transcription control of the earliest cellular events
in hematopoiesis.
TEL Gene Rearrangements in Myeloid Malignancy 1207
Todd R. Golub
The TEL gene is a recently described, promiscuous gene with
a role in both myeloid and lymphoid malignancy. It is unusual
since there may be more than one mechanism by which its re¬
arrangement through chromosomal translocation is leukemo
genic. This article discusses the four potential mechanisms of
TEL mediated transformation. It is conceivable that the TEL gene
is the common target for various translocations precisely because
of this pleiotropy of pathogenic mechanisms by which TEL gene
rearrangements can lead to cell transformation.
Hox Homeobox Genes as Regulators of Normal and
Leukemic Hematopoiesis 1221
Unnur Thorsteinsdottir, Guy Sauvageau, and
R. Keith Humphries
Hox genes, first recognized for their role in embryonic develop¬
ment, may also play lineage specific functions in a variety of
somatic tissues including the hematopoietic system. Expression
of these transcription factors has been demonstrated both in nor¬
mal and leukemic human and hematopoietic cells, suggesting
functional roles in hematopoietic cell growth and differentiation.
Several recent studies have shown that Hox proteins are involved
in controlling proliferation of primitive bone marrow cells and
viii contents
also in altering differentiation of myeloid as well as lymphoid
progenitors, alterations that also can contribute to leukemic trans¬
formation. Hox genes, together with their upstream regulators
and downstream target genes, may play key roles in fundamental
processes controlling hematopoietic stem cell properties.
Cumulative Index 1997 1239
Subscription Information Inside back cover
CONTENTS ix
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spelling | Aplastic anemia and stem cell biology Leonard I. Zon, guest ed. Philadelphia [u.a.] Saunders 1997 XIV S., S. 1025 - 1259 Ill., graph. Darst. txt rdacontent n rdamedia nc rdacarrier Hematology, oncology clinics of North America 11,6 Anémie aplasique - étiologie Cellules souches hématopoïétiques Pancytopenie gtt Stamcellen gtt Anemia, Aplastic etiology Aplastic anemia Etiology Hematopoietic Stem Cells Hematopoietic stem cells Blutstammzelle (DE-588)4146093-5 gnd rswk-swf Hämatopoese (DE-588)4113823-5 gnd rswk-swf Aplastische Anämie (DE-588)4228474-0 gnd rswk-swf (DE-588)4143413-4 Aufsatzsammlung gnd-content Aplastische Anämie (DE-588)4228474-0 s Blutstammzelle (DE-588)4146093-5 s DE-604 Hämatopoese (DE-588)4113823-5 s Zon, Leonard I. Sonstige oth Hematology, oncology clinics of North America 11,6 (DE-604)BV000625446 11,6 HBZ Datenaustausch application/pdf http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=007907239&sequence=000002&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA Inhaltsverzeichnis |
spellingShingle | Aplastic anemia and stem cell biology Hematology, oncology clinics of North America Anémie aplasique - étiologie Cellules souches hématopoïétiques Pancytopenie gtt Stamcellen gtt Anemia, Aplastic etiology Aplastic anemia Etiology Hematopoietic Stem Cells Hematopoietic stem cells Blutstammzelle (DE-588)4146093-5 gnd Hämatopoese (DE-588)4113823-5 gnd Aplastische Anämie (DE-588)4228474-0 gnd |
subject_GND | (DE-588)4146093-5 (DE-588)4113823-5 (DE-588)4228474-0 (DE-588)4143413-4 |
title | Aplastic anemia and stem cell biology |
title_auth | Aplastic anemia and stem cell biology |
title_exact_search | Aplastic anemia and stem cell biology |
title_full | Aplastic anemia and stem cell biology Leonard I. Zon, guest ed. |
title_fullStr | Aplastic anemia and stem cell biology Leonard I. Zon, guest ed. |
title_full_unstemmed | Aplastic anemia and stem cell biology Leonard I. Zon, guest ed. |
title_short | Aplastic anemia and stem cell biology |
title_sort | aplastic anemia and stem cell biology |
topic | Anémie aplasique - étiologie Cellules souches hématopoïétiques Pancytopenie gtt Stamcellen gtt Anemia, Aplastic etiology Aplastic anemia Etiology Hematopoietic Stem Cells Hematopoietic stem cells Blutstammzelle (DE-588)4146093-5 gnd Hämatopoese (DE-588)4113823-5 gnd Aplastische Anämie (DE-588)4228474-0 gnd |
topic_facet | Anémie aplasique - étiologie Cellules souches hématopoïétiques Pancytopenie Stamcellen Anemia, Aplastic etiology Aplastic anemia Etiology Hematopoietic Stem Cells Hematopoietic stem cells Blutstammzelle Hämatopoese Aplastische Anämie Aufsatzsammlung |
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