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Monoclonal Gammopathies and the Kidney -

Monoclonal Gammopathies and the Kidney

Buch | Softcover
300 Seiten
2010 | Softcover reprint of hardcover 1st ed. 2003
Springer (Verlag)
978-90-481-6199-7 (ISBN)
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Table 1 Comparison of MIDD and immunoglobulinic amyloidosis MIDD Amyloidosis Presentation Multivisceral or predominant involvement of an organ (kidney) Underlying disease Monoclonal immunoproliferative disorders, mostly myeloma. Monoclonal bone marrow plasma cell populations without overt malignancy. Possibility of 'non-secretory' forms. Deposits by EM (Granular) non-organized Fibrillar (13 pleated sheets) Spectrum LCDD : L chains, most K, either AL-amyloidosis : fragments (?)* of or apparently enlarged, normal-sized normal-sized or apparently enlarged or short. Frequent glycosylation L chains most A. Frequent and polymeration. glycosylation.Abnormal susceptibility to proteolysis. LHCDD : associated H chains Cases with associated short H chains (short by biosynthesis by biosynthesis experiments. experiments). HCDD: short H chains. AH-amyloidosis : short H chains. L chain isotypy V K IV predominance. VAVI C A3predominance. V region mutations Yes Yes *Found in necropsy material; whether they exist in vivo is doubtful. 3 After Preud'homme et al. AL-amyloidosis).
Therefore, we believe' that the term MIDD should be restricted to this pathological entity, whatever the Ig content of the deposits, light 4 5 ) or both (LHCDD ). However, the chains (LCDD), heavy chains (HCDD New York group used the denomination MIDD for both Randall's disease and immunoglobulinic amyloidosis'S". The two processes have many features in common (Table I) and they can be associated in the same patients.

I: The Culprits: the Plasma Cell Dyscrasias.- 1 Physiopathology of malignant B cell lymphoproliferations.- 2 Immunoglobulin synthesis and secretion.- 3 Immunoglobulin decreased solubility diseases: pathologies of the V domains?.- 4 Megalin, cubilin and immunoglobulin light chains: receptor-mediated uptake of light chains in kidney proximal tubule.- II: The Victim: the Tubule.- 5 Pathophysiology of myeloma kidney.- 6 Myeloma cast nephropathy: clinical presentation.- 7 New therapeutic approaches for the myeloma patient with and without renal failure.- 8 Adult Fanconi’s syndrome due to monoclonal immunoglobulin light chains: an underdiagnosed disease with unexpected clinical and pathologic heterogeneity.- 9 Monoclonal immunoglobulin light chains associated to Fanconi’s syndrome.- III: The Victim: the Glomerulus.- 10 Ultrastructural pattern and classification of renal monoclonal immunoglobulin deposits.- 11 Morphological aspects in light chain deposition disease.- 12 Renal monoclonal immunoglobulin deposition disease (MIDD): the Columbia experience.- 13 Monoclonal cryoglobulinemia kidney.- 14 Fibrillary glomerulonephritis: defining the disease spectrum.- 15 Glomerulonephritis with organized immunoglobulin deposits: fibrillar and microtubular deposits are associated with distinct immunological features.- IV: Immunoglobulins Amyloidosis.- 16 Clinical features and management of amyloidosis.- 17 Fibrillogenesis and therapy of amyloidosis: an equilibrium approach.- 18 Immunoglobulin light chain amyloidosis and the kidney.- 19 Treating primary systemic amyloidosis with stem cell transplantation: outcomes in renal amyloidosis.- 20 Prognostic factors for survival and response after high-dose therapy and autologous stem cell transplantation in systemic AL amyloidosis: a reporton 21 patients.- 21 Presentation of the French multicentre randomized trial comparing intensive and conventional treatment for AL amyloidosis.- 22 Amyloidosis: Round Table.- Oral communications.- Poster communications.

Erscheint lt. Verlag 1.12.2010
Zusatzinfo XVI, 300 p.
Verlagsort Dordrecht
Sprache englisch
Maße 155 x 235 mm
Themenwelt Medizinische Fachgebiete Innere Medizin Hämatologie
Medizinische Fachgebiete Innere Medizin Nephrologie
Studium 2. Studienabschnitt (Klinik) Pathologie
Studium Querschnittsbereiche Infektiologie / Immunologie
Naturwissenschaften Biologie Biochemie
ISBN-10 90-481-6199-1 / 9048161991
ISBN-13 978-90-481-6199-7 / 9789048161997
Zustand Neuware
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