Myeloproliferative diseases and myelodysplastic syndromes:
Gespeichert in:
| Format: | Buch |
|---|---|
| Sprache: | English |
| Veröffentlicht: |
Philadelphia [u.a.]
Saunders
2003
|
| Schriftenreihe: | Hematology, oncology clinics of North America
17,5 |
| Schlagworte: | |
| Online-Zugang: | Inhaltsverzeichnis |
| Beschreibung: | XXII S., S. 1095 - 1275 Ill., graph. Darst. |
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Datensatz im Suchindex
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| adam_text | MYELOP^UPERATIVR DISEASES A 5VKPBC9NB3S
CONTENTS
Foreword xi
Frank H. Gardner
Preface xxi
Nathanial I. Berlin
The Myelodysplastic/Myeloproliferative Disorders: The Interface 1095
John M. Bennett
Most patients with myelodysplastic syndromes can be identified eas¬
ily and separated from patients who have myeloproliferative dis¬
orders. A few have overlapping features, however, with evidence of
morphologic dysplasia and a proliferative advantage of one or more
of the myeloid differentiating cell lines. For practical purposes and
therapeutic considerations, chronic myelomonocytic leukemia is
viewed now as a composite disorder along with atypical chronic
myeloid leukemia and juvenile myelomonocytic leukemia.
Pathology of the Myeloproliferative Diseases 1101
Tracy I. George and Daniel A. Arber
The myeloproliferative diseases include chronic myeloproliferative
disorders and mixed myelodysplastic/myeloproliferative disorders.
This article reviews the pathology of myeloproliferative diseases,
including pertinent clinical, laboratory, histologic, cytogenetic, and
molecular genetic findings necessary for the classification of these
disorders. The latest classification changes are discussed as are pro¬
mising new molecular genetic findings. Chronic myeloproliferative
disorders comprise chronic myelogenous leukemia, polycythemia
vera, chronic idiopathic myelofibrosis, essential thrombocythemia,
chronic neutrophilic leukemia, chronic eosinophilic leukemia, and
unclassifiable myeloproliferative disease. Mixed myelodysplas¬
tic/myeloproliferative disorders comprise chronic myelomonocytic
VOLUME 17 • NUMBER 5 • OCTOBER 2003 v
leukemia, atypical chronic myelogenous leukemia, juvenile
myelomonocytic leukemia, and unclassifiable myelodysplas
tic/myeloproliferative disease.
Cytogenetics of Chronic Myeloprolif erative Disorders and
Related Myelodysplastic Syndromes 1129
Adewale Adeyinka and Gordon W. Dewald
The only myeloproliferative disorder associated with any specific
chromosome anomaly is chronic myeloid leukemia, which is linked
with t(9;22)(q34;qll.2) or a variant of this anomaly. An association
exists for del(13)(ql2ql4) and myelofibrosis; t(5;12)(q33;pl3) and
eosinophilia; and del(20qll), +8, and +9 and polycythemia vera, but
these anomalies can be seen in various hematologic malignancies.
The most common chromosomal anomalies among myeloprolifera¬
tive disorders in order of frequency are t(9;22)(q34;qll.2), Y, +8, +9,
7, del(20)(qllql3), del(13)(ql2ql4), del(5)(ql3q33), and del(12)(pl2).
New fluorescent labeled DNA techniques are useful for myeloprolif¬
erative disorders to study inadequate bone marrow or blood speci¬
mens and to monitor disease status among patients with known
chromosome anomalies, but they are not more sensitive than con¬
ventional chromosome studies.
Classification and Molecular Biology of Polycythemias
(Erythrocytoses) and Thrombocytosis 1151
Josef T. Prchal
In this article, polycythemic disorders are classified based on the
current understanding of biology of erythropoieses and divided
into primary and secondary polycythemias. Special emphasis is
given to recently uncovered molecular bases of newly described
congenital polycythemic disorders. This clarification of the patho
physiology of some of the congenital polycythemic states has obvi¬
ous utility for more accurate diagnosis and rational prognostic
determination. The molecular basis of congenital thrombocytoses
is only beginning to be uncovered. In contrast, the molecular bases
of polycythemia vera and essential thrombocythemia remain
unknown, thus their diagnostic criteria are imprecise and their
treatment remains largely empirical. The central premise of this
article is that deciphering the molecular basis of human diseases
leads to improved understanding of hematopoiesis, precise diag¬
nosis, and the potential for development of a specific therapy.
Chronic Myeloid Leukemia 1159
Richard T. Silver
Chronic myeloid leukemia is a clonal myeloproliferative disorder
of a pluripotent stem cell with a specific cytogenetic abnormality,
the Philadelphia chromosome, involving myeloid, erythroid,
megakaryocytic, B lymphoid, and sometimes T lymphoid cells but
vi CONTENTS
not marrow fibroblasts. Advances in cell biology and molecular
genetics and a plethora of biochemical, cy togenetic, and molecular
data of clinical relevance have yielded much new information
regarding this disease. This article reviews the hematologic and
clinical aspects of chronic myeloid leukemia; discusses the perti¬
nent aspects of the advances in understanding of the cytogenetics
and molecular biology of the disease; and reviews treatment pro¬
grams employing busulfan, hydroxyurea, interferon, and marrow
transplantation, which still are clinically important and relevant
despite the development of the exciting new drug imatinib mesyl
ate, a new paradigm for cancer chemotherapy in general.
Essential Thrombocythemia 1175
Claire N. Harrison and Anthony R. Green
Essential thrombocythemia is a distinct clinical entity within the
spectrum of myeloproliferative disorders. There is as yet no pathog
nomonic diagnostic test, and patients who currently fall into the cate¬
gory of essential thrombocythemia are likely to be heterogeneous.
This article discusses diagnostic criteria, clinical features, prognosis,
and management.
Polycythemia Vera 1191
Nathanial I. Berlin
Polycythemia vera is a clonal disorder of the pluripotent bone mar¬
row stem cell that gives rise to increased circulating red blood cells,
leucocytes, and platelets. Polycythemia vera is characterized by a
ruddy cyanosis, enlargement of the spleen in 70%, the liver in 30%,
and hypertension in 50%. The principle symptoms are fatigue,
weakness, headache, epigastric pain, and pruritis. The differential
diagnosis of an elevated hematocrit and the criteria for the diag¬
nosis of polycythemia vera present little or no problem; however,
there is not a consensus on therapy.
Myelofibrosis with Myeloid Metaplasia 1211
Giovanni Barosi
Myelofibrosis with myeloid metaplasia is a chronic myeloprolifer¬
ative disorder characterized by bone marrow fibrosis and neoan
giogenesis, constitutive release of a high number of CD34+ stem
cells from the bone marrow, and extramedullary hematopoiesis. It
presents with heterogeneous clinical features in which anemia and
progression to symptomatic splenomegaly dominate. The patho
genesis is undefined. Allogeneic stem cell transplantation offers a
chance of cure. Autologus stem cell transplantation and splenectomy
are risky procedures that may be considered in advanced disease
when conventional therapies for correcting anemia (danazol,
recombinant human erythropoietin, cyclosporine) or chemotherapy
for enlarging splenomegaly and myeloproliferation (hydroxy urea
or interferon alfa) have failed. Thalidomide has been tested in
CONTENTS vii
numerous series, and its capacity to improve anemia and throm
bocytopenia while reducing splenomegaly has been documented.
Mast Cell Proliferative Disorders: Current View on Variants
Recognized by the World Health Organization 1227
Peter Valent, Cem Akin, Wolfgang R. Sperr, Hans Peter Horny,
and Dean D. Metcalfe
Mastocytosis is a heterogeneous group of disorders characterized
by abnormal growth and accumulation of mast cells in one or more
organs. Systemic variants of disease can be regarded as myelopro
liferative disorders. Similar to other myeloid neoplasms, systemic
mastocytosis shows a variable clinical course and prognosis and
can progress into frank leukemia. The treatment of mastocytosis
must be adjusted to the individual situation in each patient and the
subtype of disease.
Allogeneic Hematopoietic Stem Cell Transplantation for
Myeloproliferative Disorders and Myelodysplastic Syndromes 1243
Alan S. Wayne and A.J. Barrett
Allogeneic hematopoietic stem cell transplantation (SCT) is the
only proven curative therapy for patients with myeloproliferative
diseases. As a stem cell disorder, chronic myelogenous leukemia
(CML) has served as a prototypic disease in the development of
allogeneic SCT. Since the initial reports of the successful use of
bone marrow transplantation for CML, the use of SCT for this dis¬
order has become standard therapy. CML currently is one of the
most common indications for allogeneic SCT, and the success of
SCT in the treatment of CML has served as a benchmark for trans¬
plantation trials in other malignancies. The curative potential of an
allogeneic graft versus leukemia effect also has been shown in
CML, which has fueled major efforts in the study and application
of this new approach to anticancer therapeutics. This article high¬
lights the principles and results of allogeneic SCT in the treatment
of CML and discusses the use of SCT for other myeloproliferative
diseases and myelodysplastic syndromes.
Myeloproliferative and Myelodysplastic Syndromes: The Future 1261
John M. Goldman
The incidence of chronic myeloproliferative diseases and myelodys¬
plastic syndromes could be reduced if relevant environmental fac¬
tors could be identified and eliminated, but this seems an unlikely
prospect for the foreseeable future. More probable is the likelihood
that the molecular basis of the chronic myeloproliferative diseases
gradually will be elucidated such that specific inhibitory molecules
analogous to imatinib may be designed for each disease or each sub¬
type. Combinations of inhibitory molecules may prove especially
useful. There is ample scope for improving the clinical results of
viii CONTENTS
allogeneic stem cell transplantation, which theoretically could
cure most patients with myeloproliferative diseases or myelodys
plastic syndromes. Reduced intensity conditioning allogeneic stem
cell transplants seem promising. One possibility is identification and
exploitation of the basic mechanism underlying the graft versus
leukemia effect for eradication of minimal residual disease without
the need for allografting.
Index 1271
CONTENTS ix J#*
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| spelling | Myeloproliferative diseases and myelodysplastic syndromes Nathanial I. Berlin guest ed. Philadelphia [u.a.] Saunders 2003 XXII S., S. 1095 - 1275 Ill., graph. Darst. txt rdacontent n rdamedia nc rdacarrier Hematology, oncology clinics of North America 17,5 Myelodysplastische syndromen gtt Myeloproliferatieve ziekten gtt Mast cell disease Myelodysplastic Syndromes Myeloproliferative Disorders Stem cells Transplantation Myelodysplastisches Syndrom (DE-588)4281183-1 gnd rswk-swf Myeloproliferatives Syndrom (DE-588)4301911-0 gnd rswk-swf (DE-588)4143413-4 Aufsatzsammlung gnd-content Myeloproliferatives Syndrom (DE-588)4301911-0 s Myelodysplastisches Syndrom (DE-588)4281183-1 s DE-604 Berlin, Nathaniel I. Sonstige oth Hematology, oncology clinics of North America 17,5 (DE-604)BV000625446 17,5 HBZ Datenaustausch application/pdf http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=010577686&sequence=000002&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA Inhaltsverzeichnis |
| spellingShingle | Myeloproliferative diseases and myelodysplastic syndromes Hematology, oncology clinics of North America Myelodysplastische syndromen gtt Myeloproliferatieve ziekten gtt Mast cell disease Myelodysplastic Syndromes Myeloproliferative Disorders Stem cells Transplantation Myelodysplastisches Syndrom (DE-588)4281183-1 gnd Myeloproliferatives Syndrom (DE-588)4301911-0 gnd |
| subject_GND | (DE-588)4281183-1 (DE-588)4301911-0 (DE-588)4143413-4 |
| title | Myeloproliferative diseases and myelodysplastic syndromes |
| title_auth | Myeloproliferative diseases and myelodysplastic syndromes |
| title_exact_search | Myeloproliferative diseases and myelodysplastic syndromes |
| title_full | Myeloproliferative diseases and myelodysplastic syndromes Nathanial I. Berlin guest ed. |
| title_fullStr | Myeloproliferative diseases and myelodysplastic syndromes Nathanial I. Berlin guest ed. |
| title_full_unstemmed | Myeloproliferative diseases and myelodysplastic syndromes Nathanial I. Berlin guest ed. |
| title_short | Myeloproliferative diseases and myelodysplastic syndromes |
| title_sort | myeloproliferative diseases and myelodysplastic syndromes |
| topic | Myelodysplastische syndromen gtt Myeloproliferatieve ziekten gtt Mast cell disease Myelodysplastic Syndromes Myeloproliferative Disorders Stem cells Transplantation Myelodysplastisches Syndrom (DE-588)4281183-1 gnd Myeloproliferatives Syndrom (DE-588)4301911-0 gnd |
| topic_facet | Myelodysplastische syndromen Myeloproliferatieve ziekten Mast cell disease Myelodysplastic Syndromes Myeloproliferative Disorders Stem cells Transplantation Myelodysplastisches Syndrom Myeloproliferatives Syndrom Aufsatzsammlung |
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