عنوان

Paroxysmal Nocturnal Hemoglobinuria and Related Disorders :

(Number (ISBN

4431678670

(Number (ISBN

9784431678670

Number

b590880

Title Proper

Paroxysmal Nocturnal Hemoglobinuria and Related Disorders :

General Material Designation

[Book]

Other Title Information

Molecular Aspects of Pathogenesis

First Statement of Responsibility

edited by Mitsuhiro Omine, Taroh Kinoshita.

Place of Publication, Distribution, etc.

Tokyo

Name of Publisher, Distributor, etc.

Springer Japan

Date of Publication, Distribution, etc.

2003

Specific Material Designation and Extent of Item

(xviii, 285 pages)

Text of Note

From the contents: I. Overview of PNH: 1. An Overview of PNH; Molecular Genetics. 2. An Overview of the Development of Specific Inhibitors of Complement: Opportunities for Therapy of PNH --; II. Pathogenesis and Animal Models of PNH: 3. Mice with Blood Cells Deficient in GPI-Linked Proteins --; the Generation of a Murine Model to Investigate the Pathogenesis and Pathophysiology of PNH. 4. A multistep Model for the Pathogenesis and Evolution of PNH. 5. Some New Viewpoints of Molecular Pathogenesis in Paroxysmal Nocturnal Hemoglobinuria (PNH) --; III. Clinical Pathology and Natural History of PNH: 6. Paroxysmal Nocturnal Hemoglobinuria: History and Pathology. 7. Long-Term Outcome in PNH; the French Society of Hematology Experience --; IV. Mechanism of Clonal Expansion in PNH: 8. PNH Clone is Less Susceptible to Killing by NK Cells. 9. The Role of Lymphoid Cell Clones in the Pathogenesis of PNH. 10. A Possible Intrinsic Mechanism for Clonal Expansion of PNH Abnormal Cells --; V. PNH Clones under Bone Marrow Failure: 11. Clinical Significance of Increased PNH-Type Cells in the Peripheral Blood of Patients with Aplastic Anemia and Refractory Anemia. 12. Incidence and Clinical Significance of PNH Clone in Myelodysplastic Syndromes (MDS). 13. The Enigmatic Relationship of Paroxysmal Noctural Hemoglobinuria and Acquired Aplastic Anemia --; VI. PNH, Aplastic Anemia, Myelodysplastic Syndromes: (1) Pathology: 14. Does PNH Ever Arise De Novo? 15. Telomeres in Myelodysplastic Syndromes. 16. Immunosuppression in Aplastic Anemia. (2) Clinical Aspects: 17. MDS/Leukemia and PNH After Immunosuppressive Therapy for Severe Aplastic Anemia. 18. Stem Cell Transplantation in Aplastic Anemia and PNH: Similarities and Diversities --; VII. Related Topics.

Text of Note

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.

Hematology.

Medicine.

Class number

Book number

edited by Mitsuhiro Omine, Taroh Kinoshita.

Mitsuhiro Omine

Taroh Kinoshita

Electronic name

[Book]

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