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Blood, 15 October 2006, Vol. 108, No. 8, pp. 2520-2530. Prepublished online as a Blood First Edition Paper on June 22, 2006; DOI 10.1182/blood-2006-03-001164. This Article Full Text Full Text (PDF) All Versions of this Article: blood-2006-03-001164v1 108/8/2520    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Merlini, G. Articles by Stone, M. J. Search for Related Content PubMed PubMed Citation Articles by Merlini, G. Articles by Stone, M. J. Related Collections Immunobiology Neoplasia Reviews in Translational Hematology Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  REVIEW IN TRANSLATIONAL HEMATOLOGY Dangerous small B-cell clones Giampaolo Merlini, and Marvin J. Stone From the Amyloid Center, Biotechnology Research Laboratories, Foundation Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Policlinico San Matteo, Department of Biochemistry, University of Pavia, Italy; and the Baylor Charles A. Sammons Cancer Center and Immunology Laboratory, Baylor University Medical Center, Dallas, TX. The detection of a monoclonal immunoglobulin in serum or urine usually raises concerns about the size of the underlying B-cell-derived clone and possible systemic effects caused by its expansion. However, a small clone can synthesize a very toxic protein, producing devastating systemic damage and protean clinical presentations. The resulting "monoclonal component-related diseases," although difficult to diagnose, may be progressive and even fatal. The monoclonal protein can aggregate and deposit systemically as occurs in light-chain amyloidosis, monoclonal immunoglobulin deposition disease, crystal-storing histiocytosis, and monoclonal cryoglobulinemia. Alternatively, some monoclonal proteins possess antibody activity toward autogenous antigens and cause chronic cold agglutinin disease, mixed cryoglobulinemia, and peripheral neuropathies. Other humoral mediators may contribute to neuropathy in variant disorders such as the POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) syndrome. The clone synthesizing the noxious monoclonal proteins is often small, and sensitive techniques may be required to detect these immunoglobulins. A delay in diagnosis can allow irreversible organ damage and dramatically shorten survival. Prompt recognition of suggestive signs and symptoms should trigger a thorough diagnostic approach to reach the correct diagnosis quickly, because this is the key to effective therapy. Although the treatment of these conditions is not optimal, significant advances have been made, improving the duration and quality of life. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: S. Vanderschueren, M. Mylle, D. Dierickx, M. Delforge, P. Verhamme, K. Peerlinck, W. Meersseman, and D. C. Knockaert Monoclonal Gammopathy of Undetermined Significance: Significant Beyond Hematology Mayo Clin. Proc., September 1, 2009; 84(9): 842 - 845. [Full Text] [PDF] D. E. Reece, V. Sanchorawala, U. Hegenbart, G. Merlini, G. Palladini, J.-P. Fermand, R. A. Vescio, X. Liu, Y. A. Elsayed, A. Cakana, et al. Weekly and twice-weekly bortezomib in patients with systemic AL amyloidosis: results of a phase 1 dose-escalation study Blood, August 20, 2009; 114(8): 1489 - 1497. [Abstract] [Full Text] [PDF] G. Palladini and G. Merlini Current treatment of AL amyloidosis Haematologica, August 1, 2009; 94(8): 1044 - 1048. [Full Text] [PDF] G. Palladini, P. Russo, T. Bosoni, L. Verga, G. Sarais, F. Lavatelli, M. Nuvolone, L. Obici, S. Casarini, S. Donadei, et al. Identification of Amyloidogenic Light Chains Requires the Combination of Serum-Free Light Chain Assay with Immunofixation of Serum and Urine Clin. Chem., March 1, 2009; 55(3): 499 - 504. [Abstract] [Full Text] [PDF] S. Kumar, A. Dispenzieri, M. A. Gertz, J. Mehta, H. J. Lachmann, A. D. Wechalekar, J. D. Gillmore, H. M. Lokhorst, B. P.C. Hazenberg, A. Croockewit, et al. High-Dose Melphalan versus Melphalan plus Dexamethasone for AL Amyloidosis N. Engl. J. Med., January 3, 2008; 358(1): 91 - 93. [Full Text] [PDF] R. Sitia, G. Palladini, and G. Merlini Bortezomib in the treatment of AL amyloidosis: targeted therapy? Haematologica, October 1, 2007; 92(10): 1302 - 1307. [Full Text] [PDF] E. Kastritis, A. Anagnostopoulos, M. Roussou, S. Toumanidis, C. Pamboukas, M. Migkou, A. Tassidou, I. Xilouri, S. Delibasi, E. Psimenou, et al. Treatment of light chain (AL) amyloidosis with the combination of bortezomib and dexamethasone Haematologica, October 1, 2007; 92(10): 1351 - 1358. [Abstract] [Full Text] [PDF] V. Rosti, M. Massa, R. Campanelli, M. De Amici, G. Piccolo, and V. Perfetti Vascular endothelial growth factor promoted endothelial progenitor cell mobilization into the peripheral blood of a patient with POEMS syndrome Haematologica, September 1, 2007; 92(9): 1291 - 1292. [Abstract] [Full Text] [PDF] L. Cook and D. H C Macdonald Management of paraproteinaemia Postgrad. Med. J., April 1, 2007; 83(978): 217 - 223. [Abstract] [Full Text] [PDF] G. P. Schechter A triple on AL amyloidosis, waiting for a home run Blood, January 15, 2007; 109(2): 391 - 392. [Full Text] [PDF]

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