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Titlebook: Paroxysmal Nocturnal Hemoglobinuria and Related Disorders; Molecular Aspects of Mitsuhiro Omine (Professor),Taroh Kinoshita (Profe Conferen

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发表于 2025-3-21 18:29:16 | 显示全部楼层 |阅读模式
书目名称Paroxysmal Nocturnal Hemoglobinuria and Related Disorders
副标题Molecular Aspects of
编辑Mitsuhiro Omine (Professor),Taroh Kinoshita (Profe
视频video
概述Provides an invaluable summary of current research on the fundamental aspects of PNH pathology, presented by renowned experts in the field
图书封面Titlebook: Paroxysmal Nocturnal Hemoglobinuria and Related Disorders; Molecular Aspects of Mitsuhiro Omine (Professor),Taroh Kinoshita (Profe Conferen
描述.Few publications focus on the mysterious, genetically acquired disease paroxysmal nocturnal hemoglobinuria (PNH) and the related "intractable" disorders—aplastic anemia and myelodysplastic syndromes. Now, however, the latest understanding of the clinical and molecular genetic aspects of PNH is summarized here in the proceedings of the International Symposium held in Tokyo in 2001. Major topics reviewed include the molecular mechanisms of the PIG-A gene mutation; complement activation and inhibitors; experimental animal models; pathogenesis; the history of PNH research; the natural history of the disease; the mechanism of PNH clone expansion; the emergence of PNH clones under bone marrow failure syndromes; and treatment of the disease by immunosuppressive agents and stem cell transplantation. This book provides an invaluable summary of current research on the fundamental aspects of PNH pathology, presented by renowned experts in the field. ... .
出版日期Conference proceedings 2003
关键词anemia; blood; bone marrow; stem cell transplantation; transplantation
版次1
doihttps://doi.org/10.1007/978-4-431-67867-0
isbn_softcover978-4-431-68004-8
isbn_ebook978-4-431-67867-0
copyrightSpringer Japan 2003
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发表于 2025-3-21 23:39:27 | 显示全部楼层
A Multistep Model for the Pathogenesis and Evolution of PNHransformation of PNH into myelodysplasia or leukemia are just several aspects of PNH that are poorly understood. Previous models for the development of PNH are somewhat simplistic and do not address each of these important questions. In this report, we suggest a new multistep model for the pathogenesis and evolution of PNH.
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发表于 2025-3-22 06:04:12 | 显示全部楼层
Proposals for Classification of the Clinical Stages, Grading of Severity and the Molecular Pathogenesis of Paroxysmal Nocturnal Hemoglobinuriad manner plays an important role in the occurrence of a PNH clone, and that a PNH clone increases or decreases relatively or absolutely through competition between proliferation of WT1-expressing cells and clonal selection against WT1-expressing cells.
发表于 2025-3-22 10:22:02 | 显示全部楼层
PNH clone acquires both a survival and a growth advantage?clonal expansion. Indeed, we have detected a preferential hematopoiesis by PNH stem cells when transplanted in mice, supporting the intrinsic growth alteration. Thus, we conclude that PNH clone acquires both a growth and a survival advantage.
发表于 2025-3-22 15:32:12 | 显示全部楼层
The Role of Lymphoid Cells in the Pathogenesis of PNH attack, survive and expand. However, the mechanism responsible for the bone marrow failure and the target of the autoaggressive T cells remains still unknown. Here, we review the the most recent findings and possible mechanisms.
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发表于 2025-3-23 00:03:17 | 显示全部楼层
Overview of Paroxysmal Nocturnal Hemoglobinuria: Molecular Geneticswith aplastic anemia have clearly recognizable PIG-A mutant clone. These findings suggest that somatic mutation of PIG-A occurs in normal individuals and that clonal expansion requires extra event(s), such as selection by some pathological condition and/or some additional genetic change.
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发表于 2025-3-23 05:34:28 | 显示全部楼层
Clinical Pathology and natural history of PNH; The French Society of Hematology experience.rogens led to an increased risk of death (∼ 5 fold) in patients with . PNH while it lowered the risk of death (∼ 4 fold) in patients with the AA-PNH syndrome, compared to patients treated by androgens in either group.
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