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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.
Contents
List of Principal Authors. Preface: Kidney disease-inducing monoclonal immunoglobulins; J-L. Preud'Homme. Part I: The Culprits: The Plasma Cell Dyscrasias. 1. Physiopathology of malignant B cell lymphoproliferations; K. Lassoued, D. Ghez. 2. Immunoglobulin synthesis and secretion; D.O. Beenhouwer, K.R. Chintalacharuvu, S.L. Morrison. 3. Immunoglobulin decreased solubility diseases: pathologies of the V domains? M. Cogné, H.R. Galea, C. Sira, C. Decourt. 4. Megalin, cubilin and immunoglobulin light chains; H. Birn, M. Leboulleux, S.K. Moestrup, P.M. Ronco, P. Aucouturier, E.L. Christensen. Part II: The Victim: The Tubule. 5. Pathophysiology of myeloma kidney; P.W. Sanders. 6. Myeloma cast nephropathy: clinical presentation; A. Hummel, P. Lesavre, H.-H. Noël, D. Droz, P. Aucouturier, D. Chauveau, M. Touam, J.-P. Grünfeld. 7. New therapeutic approaches of the myeloma patient with and without renal failure; J.P. Fermand. 8. Adult Fanconi syndrome due to monoclonal immunoglobulin light chains: an underdiagnosed disease with unexpected clinical and pathologic heterogeneity; P. Ronco, B. Mougenot, B. Moulin, M. Bouiller, T. Messiaen, P. Aucouturie. 9. Monoclonal immunoglobulin light chains associated to Fanconi syndrome; P. Aucouturier, S. Déret, M. Leboulleux, P. Ronco. Part III: The Victim: The Glomerulus. 10. Ultrastructural pattern and classification of renal monoclonal immunoglobulin deposit; G. Touchard. 11. Morphological aspects in light chain deposition diseas; F. Ferrario, S. Curioni, G. Arrigo, C. Pozzi, G. Banfi. 12. Renal monoclonalimmunoglobulin deposition disease (MIDD): The Columbia experience; J. Lin, G.S. Markowitz, A.M. Valeri, N. Kambham, W.H. Sherman, G.B. Appel, V.D. D'Agati. 13. Monoclonal cryoglobulinemia kidney; G. D'Amico. 14. Fibrillary glomerulonephritis: defining the disease spectrum; V.D. D'Agati, G.B. Appel, G.S. Markowitz, L. Truong, S. Seshan, D. Un Kim, G. Sacchi. 15. Glomerulonephritis with organized immunoglobulin deposits: fibrillar and microtubular deposits are associated with distinct immunological features; F. Bridoux, O. Coldefy, J.-M. Goujon, M. Bauwens, A. Sechet, J.-L. Preud'Homme, G. Touchard. Part IV: Immunoglobulinic Amyloidosis. 16. Clinical features and management of amyloidosis; P.N. Hawkins. 17. Fibrillogenesis and therapy of amyloidosis; J.S. Wall, M. Schell, R. Hrncic, F.J. Stevens, A. Solomon. 18. Immunoglobulin light chain amyloidosis and the kidney; M.A. Gertz, M.Q. Lac, A. Dispenzieri. 19. Treating primary systemic amyloidosis with stem cell transplantation: outcomes in renal amyloidosis; R.L. Comenzo. 20. Prognostic factors for survival and response after high-dose therapy and autologous stem cell transplantation in systemic al amyloidosis: a report on 21 patients; P. Moreau, V. Leblond, P. Bourquelot, T. Facon, A. Huynh, D. Caillot, O. Hermine, M. Attal, M. Hamidou, G. Nedellec, A. Ferrant, B. Audhuy, R. Bataille, N. Milpied, J.-L. Harousseau. 21. Presentation of the French multicentre randomized trial comparing intensive and conventional treatment for AL amyloidosis; A. Jaccard. 22. Amyloidosis; J.P. Fermand, A. Jaccard, O. Hermine. Oral Communications.
