Abstract
Light/heavy chain deposition disease (LHCDD) is a disorder with predominantly renal manifestations, though sometimes associated with systemic involvement. While the recognition of LHCDD in the kidney by pathologists has increased considerably in the last decade, that is not the case when other organs are involved. If the patient has already a diagnosis of LCHDD, then the index of suspicion is raised and recognition of extrarenal manifestations is markedly improved. This chapter highlights diagnostic criteria, challenges, and pitfalls in the differential diagnosis of LHCDD in non-renal sites. It also discusses pathogenic mechanisms associated with organ pathology.
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References
Fernand J-P, Brouet J-C. Heavy-chain diseases. Hematol Oncol Clin N Am. 1999;13:1281–94.
Ronco P, Plaisier E, Mougenot B, Aucouturier P. Immunoglobulin light (heavy)-chain deposition disease: from molecular medicine to pathophysiology-driven therapy. Clin J Am Soc Nephrol. 2006;1:1342–50.
Ronco PM, Alyanakian M-A, Mougenot B, et al. Light chain deposition disease: a model of glomerulosclerosis defined at the molecular level. J Am Soc Nephrol. 2001;12:1558–65.
Kambham N, Markowitz GS, Appel GB, et al. Heavy chain deposition disease: the disease spectrum. Am J Kidney Dis. 1999;33:954–62.
Buxbaum J, Gallo G. Nonamyloidotic monoclonal immunoglobulin deposition diseases. Hematol Oncol Clin North Am. 1999;13:1235–48.
Lin J, Markowitz GS, Valeri AM, Kambham N, et al. Renal monoclonal immunoglobulin deposition disease: the disease spectrum. J Am Soc Nephrol. 2001;12:1482–92.
Herrera GA, Joseph L, Gu X, et al. Renal pathologic spectrum in an autopsy series of patients with plasma cell dyscrasias. Arch Pathol Lab Med. 2004;128:875–9.
Nasr SH, Valeri AM, Cornell LD, Fidler ME, et al. Renal monoclonal immunoglobulin deposition disease: A report of 64 patients from a single institution. Clin J Am Soc Nephrol. 2012;7:231–9.
Ivanyi B. Frequency of light chain nephropathy relative to renal amyloidosis and Bence Jones cast nephropathy in a necropsy study of patients with myeloma. Arch Pathol Lab Med. 1990;114:986–7.
Khamlichi AA, Aucouturier P, Preud’Homme JL, Cogne M, et al. Structure of abnormal heavy chains in human heavy-chain deposition disease. Eur J Biochem. 1995;229:54–60.
Cogne M, Silvain C, Khamlichi AS, et al. Structurally abnormal immunoglobulins in human lymphoproliferative disorders. Blood. 1992;79:2181–95.
Preud’homme JL, Aucouturiere P, Touchard G, et al. Monoclonal immunoglobulin deposition disease: a review of immunoglobulin chain alterations. Int J Immunopharmacol. 1994;16:425–31.
Khamlichi AA, Rocca A, Touchard G, et al. Role of light chain variable region in myeloma with light chain deposition disease: evidence from experimental model. Blood. 1995;86:3655–9.
Turner M. Molecules which recognize antigen. In: Roitt IM, Brostoff J, Male DK, editors. Immunology. St Louis, MO: Mosby; 1989. p. 5.1–5.11.
Santostefano M, Zanchelli F, Zaccaria A, et al. The ultrastructural basis of renal pathology in monoclonal gammopathies. J Nephrol. 2005;18:659–75.
Herrera GA. The contributions of electron microscopy to the understanding and diagnosis of plasma cell dyscrasia-related renal lesions. Med Electron Microsc. 2001;34:1–18.
Gallo G, Picken MM, Buxbaum J, et al. Deposits in monoclonal immunoglobulin deposition disease lack amyloid P component. Mod Pathol. 1988;1:453–6.
Antonovych TT, Lin RC, Parrish E. Light chain deposits in multiple myeloma. 7th Annual Meeting of the American Society of Nephrology, 173 (Abstract #3). Lab Invest. 1974; 30: 370A.
Randall RE, Williamson WC, Mullinax F, et al. Manifestations of systemic light chain deposition. Am J Med. 1976;60:293–9.
Husby G, Blichfeld P, Brinch L, et al. Chronic arthritis and gamma heavy chain disease: coincidence or pathogenic link? Scand J Rheumatol. 1998;27:257–64.
Husby G. Is there a pathogenetic link between gamma heavy chain disease and chronic arthritis? Curr Opin Rheumatol. 2000;12:65–70.
Rott T, Vizjak A, Lindic J, et al. IgG heavy-chain deposition disease affecting both kidneys, skin, and skeletal muscle. Nephrol Dial Transplant. 1998;13:1825–8.
Aucouturier P, Khamlichi AA, Touchard G, et al. Brief report: heavy chain deposition disease. N Engl J Med. 1993;329:1389–93.
Hendershot I, Bole D, Kearney JF. Assembly and secretion of heavy chains that do not associate post-translationally with immunoglobulin heavy chain binding protein. J Cell Biol. 1987;104:761–7.
Cohen JJ. Case records of the Massachusetts General Hospital. Weekly clinicopathologic exercises. Case 1–1981. N Engl J Med. 1981;304:33–43.
Linder J, Croker BP, Vollmer RT, et al. Systemic kappa light-chain deposition. An ultrastructural study. Am J Surg Pathol. 1983;7:85–93.
Herrera GA. Renal manifestations in plasma cell dyscrasias: an appraisal from the patients’ bedside to the research laboratory. Ann Diagn Pathol. 2000;4:174–200.
Herrera GA. Renal lesions associated with plasma cell dyscrasias: practical approach to diagnosis, new concepts, and challenges. Arch Pathol Lab Med. 2009;133:249–67.
Ganeval D, Mignon F, Preud’homme JL, et al. Visceral deposition of monoclonal light chains and immunoglobulins: a study of renal and immunopathologic abnormalities. Adv Nephrol Necker Hosp. 1982;11:25.
Herrera GA, Turbat-Herrera EA, Viale G, et al. Ultrastructural immunolabeling in renal diseases: past, present and future expectations. Pathol Immunopathol Res. 1987;6:51–63.
Girelli CM, Lodi G, Rocca F. K Light chain deposition disease of the liver. Eur J Gastroenterol Hepatol. 1998;10:429–30.
Katz A, Zent R, Bargman JM. IgG heavy-chain deposition disease. Mod Pathol. 1994;7:874.
Droz D, Noel LH, Carnot F, et al. Liver involvement in nonamyloid light chain deposition disease. Lab Invest. 1984;50:683–9.
Ganeval D, Noel LH, Preud’homme JL, et al. Light chain deposition disease: Its relation to AL-type amyloidosis. Kidney Int. 1984;26:1–9.
Jego P, Paillard F, Ramée MP, Grosbois B. Congestive heart failure: revealing light chain deposition disease. Eur J Intern Med. 2000;11:101–3.
Fabbian F, Stabellini N, Sartori S, et al. Light chain deposition disease presenting as paroxysmal atrial fibrillation: a case report. J Med Case Rep. 2007;1:187–93.
Fisher L, Korfel A, Stoltenburg-Didinger G, et al. A 19-year-old male with generalized seizures, unconsciousness and a deviation of gaze. Brain Pathol. 2006;16:185–6.
Popovic M, Tavcar R, Glavac D, et al. Light chain deposition disease restricted to the brain: the firs case report. Hum Pathol. 2007;38:179–84.
Kijner CH, Yousem SA. Systemic light chain deposition disease presenting as multiple pulmonary nodules. A case report and review of the literature. Am J Surg Pathol. 1988;12:401–13.
Bhargava P, Rushin JM, Rusnok EJ, et al. Pulmonary light chain deposition disease. Report of five cases and review of the literature. Am J Surg Pathol. 2007;31:267–76.
Chen KT. Amyloidosis presenting in the respiratory tract. Pathol Ann. 1989;24:253–73.
Colby T, Kos MN, Travis WD. Tumors of lower respiratory tract. Atlas of tumor pathology. Washington, DC: American Registry of Pathology; 1995. p. 495–501.
Colombat M, Stern M, Groussard O, et al. Pulmonary cystic disease related to light chain deposition disease. Am J Respir Crit Care Med. 2006;173:777–80.
Morinaga S, Watanabe H, Gemma A, et al. Plasmacytoma of the lung associated with nodular deposits of immunoglobulins. Am J Surg Pathol. 1987;11:989–9995.
Warfel K, Benson MD, Hull MT, et al. Pulmonary nodules and pleural plaques in systemic light chain deposition disease (LCDD). Lab Invest. 1987;500:84a.
Khoor A, Myers JL, Tazelaar HD, et al. Amyloid-like pulmonary nodules, including localized light chain deposition: clinicopathologic analysis of three cases. Am J Clin Pathol. 2004;121:200–4.
Morigana S, Watanabe H, Gemma A, et al. Plasmacytoma of the lung with nodular deposits of immunoglobulin. Am J Surg Pathol. 1987;11:989–95.
Piard F, Yaziji N, Jarry O, et al. Solitary plasmacytoma of the lung with light chain extracellular deposits: a case report and review of the literature. Histopathology. 1998;32:356–61.
Stokes MB, Jagirdar J, Burchstin O, et al. Nodular pulmonary immunoglobulin light chain deposits with coexistent amyloid and non-amyloid features in an HIV-infected patient. Mod Pathol. 1997;10:1059–65.
Russell WJ, Cardelli J, Harris E, et al. Monoclonal light chain-mesangial cell interactions: early signaling events and subsequent pathologic effects. Lab Invest. 2001;81:689–703.
Zhu L, Herrera GA, Murphy-Ullrich JE, et al. Pathogenesis of glomerulosclerosis in light chain deposition disease. Am J Pathol. 1995;147:375–85.
Keeling J, Herrera GA. An in vitro model of light chain deposition disease. Kidney Int. 2009;75:634–45.
Teng J, Zhang PL, Russell WJ, et al. Insights into mechanisms responsible for mesangial alterations associated with fibrogenic glomerulopathic light chains. Nephron Physiol. 2003;94:28–38.
Keeling J, Teng J, Herrera GA. AL-amyloidosis and light chain deposition disease light chains induce divergent phenotypic transformations of human mesangial cells. Lab Invest. 2004;84:1322–38.
Ronco P, Plaisier E, Aucouturier P. Monoclonal immunoglobulin light and heavy chain deposition diseases: molecular models of common diseases. In: Ronco C, editor, Herrera G, editor. Experimental models of renal diseases Pathogenesis and diagnosis contributions to nephrology. Basel: Karger; 2011. p. 220–31.
Trinkaus-Randall V, Walsh MT, Steeves S, et al. Cellular response of cardiac fibroblasts to amyloidogenic light chains. Am J Pathol. 2005;106:197–208.
Picken MM, Frangione B, Barlogie B, Luna M, Gallo G. Light chain deposition disease derived from the kappa I light chain subgroup. Biochemical characterization. Am J Pathol. 1989;134:749–54.
Rocca A, Khamlichi AA, Noël L-H, et al. Primary structure of a variable region of the VκI subgroup (ISE) in light chain deposition disease. Clin Exp Immunol. 1993;91:506–9.
Decourt C, Cogne M, Rocca A. Structural peculiarities of a truncated VκIII immunoglobulin light chain in myeloma with light chain deposition disease. Clin Exp Immunol. 1996;106:357–61.
Gu M, Wilton R, Stevens FJ. Diversity and diversification of light chains in myeloma: the specter of amyloidogenesis by proxy. In: Herrera GA, editor. The kidney in plasma cell dyscrasias, Contributions to Nephrology series. S Karger AG: Basel; 2007. p. 156–81.
Jacquot C, Saint-Andre JP, Touchard G, et al. Association of systemic light chain deposition disease and amyloidosis: a report of 3 patients with renal involvement. Clin Nephrol. 1985;24:93–8.
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Herrera, G.A., Turbat-Herrera, E.A. (2015). Light/Heavy Chain Deposition Disease as a Systemic Disorder. In: Picken, M., Herrera, G., Dogan, A. (eds) Amyloid and Related Disorders. Current Clinical Pathology. Humana Press, Cham. https://doi.org/10.1007/978-3-319-19294-9_10
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DOI: https://doi.org/10.1007/978-3-319-19294-9_10
Publisher Name: Humana Press, Cham
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