Abstract
Light chain deposition disease (LCDD) is a rare disorder associated with a clonal proliferation of plasma cells, which synthesize abnormal monoclonal immunoglobulin light chains. It is characterized by systemic deposition of light chains in various organs, with the kidneys being most commonly affected. There have been few reports of isolated LCDD, i.e. in the brain, lungs and cervical lymph nodes. We here report on another patient with an isolated form of LCDD, which was limited to the pharyngeal mucosa and was associated with an extraosseus plasmacytoma of the pharynx, expanding the spectrum that has been recognized for LCDD. The patient was treated by local radiotherapy, with an excellent response. A less aggressive clinical course can probably be expected than in the usual form of LCDD, but a long-term follow-up is necessary to establish the clinical significance of this variant of LCDD.
References
Lin J, Markowitz GS, Valeri AM, Kambham N, Sherman WH, Appel GB et al (2001) Renal monoclonal immunoglobulin deposition disease: the disease spectrum. J Am Soc Nephrol 12:1482–1492
Pozzi C, Locatelli F (2002) Kidney and liver involvement in monoclonal light chain disorders. Semin Nephrol 22:319–330
Pozzi C, D’Amico M, Fogazzi GB, Curioni S, Ferrario F, Pasquali S et al (2003) Light chain deposition disease with renal involvement: clinical characteristics and prognostic factors. Am J Kidney Dis 42:1154–1163
McKenna RW, Kyle RA, Kuehl WM, Grogan TM, Harris NL, Coupland RW (2008) Plasma cell neoplasms. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW (eds) WHO classification of tumours of haemopoietic and lymphoid tissues, 2nd edn. IARC, Lyon, pp 200–213
Popović M, Tavčar R, Glavač D, Volavšek M, Pirtošek Z, Vizjak A (2007) Light chain deposition disease restricted to the brain: the first case report. Hum Pathol 38:179–184
Piard F, Yaziji N, Jarry O, Assem M, Martin L, Bernard A et al (1998) Solitary plasmacytoma of the lung with light chain extracellular deposits: a case report and review of the literature. Histopathol 32:356–361
Rostagno A, Frizzera G, Ylagan L, Kumar A, Ghiso J, Gallo G (2002) Tumoral non-amyloidotic monoclonal immunoglobulin light chain deposits (‘aggregoma’): presenting feature of B-cell dyscrasia in three cases with immunohistochemical and biochemical analyses. Br J Haematol 119:62–69
Bellotti V, Merlini G (1996) Toward understanding the molecular pathogenesis of monoclonal immunoglobulin light-chain deposition. Nephrol Dial Transplant 11:1708–1711
Randall RE, Williamson WC Jr, Mullinax F, Tung NY, Still WJ (1976) Manifestations of systemic light chain deposition. Am J Med 60:293–299
Buxbaum JN, Genega EM, Lazowski P, Kumar A, Tunick PA, Kronzon I et al (2000) Infiltrative nonamyloidotic monoclonal immunoglobulin light chain cardiomyopathy: an underappreciated manifestation of plasma cell dyscrasias. Cardiology 93:220–228
Toor AA, Ramdane BA, Joseph J, Thomas M, O’Hara C, Barlogie B et al (2006) Cardiac nonamyloidotic immunoglobulin deposition disease. Mod Pathol 19:233–237
Rivest C, Turgeon PP, Senécal JL (1993) Lambda light chain deposition disease presenting as an amyloid-like arthropathy. J Rheumatol 20:880–884
Bhargava P, Rushin JM, Rusnock EJ, Hefter LG, Franks TJ, Sabnis SG et al (2007) Pulmonary light chain deposition disease: report of five cases and review of the literature. Am J Surg Pathol 31:267–276
Colombat M, Mal H, Copie-Bergman C, Diebold J, Damotte D, Callard P et al (2008) Primary cystic lung light chain deposition disease: a clinicopathologic entity derived from unmutated B cells with a stereotyped IGHV4-34/IGKV1 receptor. Blood 112:2004–2012
Khoor A, Myers JL, Tazelaar HD, Kurtin PJ (2004) Amyloid-like pulmonary nodules, including localized light-chain deposition: clinicopathologic analysis of three cases. Am J Clin Pathol 121:200–204
Weichman K, Dember LM, Prokaeva T, Wright DG, Quillen K, Rosenzweig M et al (2006) Clinical and molecular characteristics of patients with non-amyloid light chain deposition disorders, and outcome following treatment with high-dose melphalan and autologous stem cell transplantation. Bone Marrow Transplant 38:339–343
Lorenz EC, Gertz MA, Fervenza FC, Dispenzieri A, Lacy MQ, Hayman SR et al (2008) Long-term outcome of autologous stem cell transplantation in light chain deposition disease. Nephrol Dial Transplant 23:2052–2057
Dores GM, Landgren O, McGlynn KA, Curtis RE, Linet MS, Devesa SS (2009) Plasmacytoma of bone, extramedullary plasmacytoma, and multiple myeloma: incidence and survival in the United States, 1992–2004. Br J Haematol 144:86–94
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Zidar, N., Zver, S. & Jurčić, V. Extraosseus Plasmacytoma of the Pharynx with Localized Light Chain Deposition. Case Report. Pathol. Oncol. Res. 16, 249–252 (2010). https://doi.org/10.1007/s12253-009-9218-1
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12253-009-9218-1