Abstract
Light chain deposition disease (LCDD) is a rare disease for which an optimal treatment is not yet available. Here, we report the clinical course of a 32-year-old woman with LCDD who was successfully treated with thalidomide. She presented with nephrotic syndrome. Based on the renal biopsy findings and the presence of monoclonal immunoglobulin light chains in her serum and urine, LCDD was diagnosed. Prednisolone and cytotoxic chemotherapy used for multiple myeloma proved ineffective. We initiated administration of thalidomide (100 mg daily) and dexamethasone (20 mg for 4 days per month). After 8 months of treatment, she achieved complete hematological remission, defined as the disappearance of monoclonal protein and a normalized free light chain ratio, which led to improvement of her renal insufficiency. She has shown sustained hematological and organ response for 31 months with thalidomide therapy. Thus, thalidomide therapy seems to be a promising approach to the treatment of LCDD.
This is a preview of subscription content, log in via an institution to check access.
References
Randall RE, Williamson WC Jr, Mullinax F, Tung MY, Still WJ. Manifestations of systemic light chain deposition. Am J Med. 1976;60:293–9.
Buxbaum J. Mechanisms of disease: monoclonal immunoglobulin deposition. Amyloidosis, light chain deposition disease, and light and heavy chain deposition disease. Hematol Oncol Clin North Am. 1992;6:323–46.
Pozzi C, D’Amico M, Fogazzi GB, Curioni S, Ferrario F, Pasquali S, et al. Light chain deposition disease with renal involvement: clinical characteristics and prognostic factors. Am J Kidney Dis. 2003;42:1154–63.
Preud’homme JL, Aucouturier P, Touchard G, Striker L, Khamlichi AA, Rocca A, et al. Monoclonal immunoglobulin deposition disease (Randall type). Relationship with structural abnormalities of immunoglobulin chains. Kidney Int. 1994;46:965–72.
Heilman RL, Velosa JA, Holley KE, Offord KP, Kyle RA. Long-term follow-up and response to chemotherapy in patients with light-chain deposition disease. Am J Kidney Dis. 1992;20:34–41.
Singhal S, Mehta J, Desikan R, Ayers D, Roberson P, Eddlemon P, et al. Antitum or activity of thalidomide in refractory multiple myeloma. N Engl J Med. 1999;341:1565–71.
Barlogie B, Shaughnessy J, Tricot G, Jacobson J, Zangari M, Anaissie E, et al. Treatment of multiple myeloma. Blood. 2004;103:20–32.
Seldin DC, Choufani EB, Dember LM, Wiesman JF, Berk JL, Falk RH, et al. Tolerability and efficacy of thalidomide for the treatment of patients with light chain-associated (AL) amyloidosis. Clin Lymphoma. 2003;3:241–6.
Palladini G, Perfetti V, Perlini S, Obici L, Lavatelli F, Caccialanza R, et al. The combination of thalidomide and intermediate-dose dexamethasone is an effective but toxic treatment for patients with primary amyloidosis (AL). Blood. 2005;105:2949–51.
Barlogie B, Smith L, Alexanian R. Effective treatment of advanced multiple myeloma refractory to alkylating agents. N Engl J Med. 1984;310:1353–6.
Lazzarino M, Corso A, Barbarano L, Alessandrino EP, Cairoli R, Pinotti G, et al. DCEP (dexamethasone, cyclophosphamide, etoposide, and cisplatin) is an effective regimen for peripheral blood stem cell collection in multiple myeloma. Bone Marrow Transplant. 2001;28:835–9.
Royer B, Arnulf B, Martinez F, Roy L, Flageul B, Etienne I, et al. High dose chemotherapy in light chain or light and heavy chain deposition disease. Kidney Int. 2004;65:642–8.
Firkin F, Hill PA, Dwyer K, Gock H. Reversal of dialysis-dependent renal failure in light-chain deposition disease by autologous peripheral blood stem cell transplantation. Am J Kidney Dis. 2004;44:551–5.
Weichman K, Dember LM, Prokaeva T, Wright DG, Quillen K, Rosenzweig M, et al. 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. 2006;38:339–43.
Lorenz EC, Gertz MA, Fervenza FC, Dispenzieri A, Lacy MQ, Hayman SR, et al. Long-term outcome of autologous stem cell transplantation in light chain deposition disease. Nephrol Dial Transplant. 2008;23:2052–7.
Hassoun H, Flombaum C, D’Agati VD, Rafferty BT, Cohen A, Klimek VM, et al. High-dose melphalan and auto-SCT in patients with monoclonal Ig deposition disease. Bone Marrow Transplant. 2008;42:405–12.
Eriksson T, Hoglund P, Turesson I, Waage A, Don BR, Vu J, et al. Pharmacokinetics of thalidomide in patients with impaired renal function and while on and off dialysis. J Pharm Pharmacol. 2003;55:1701–6.
Kastritis E, Migkou M, Gavriatopoulou M, Zirogiannis P, Hadjikonstantinou V, Dimopoulos MA. Treatment of light chain deposition disease with bortezomib and dexamethasone. Haematologica. 2009;94:300–2.
San-Miguel JF, Mateos MV. How to treat a newly diagnosed young patient with multiple myeloma. Hematol Am Soc Hematol Educ Program. 2009;1:555–65.
Acknowledgments
We thank Dr. Toyokazu Yoshioka (Nishikyoto Hospital, Kyoto) for performing the renal biopsy and providing critical data of the patient, and Dr. Kazuyuki Shimizu (Nagoya City Midori General Hospital, Nagoya) for critical reading of the manuscript.
Author information
Authors and Affiliations
Corresponding author
About this article
Cite this article
Fujita, H., Hishizawa, M., Sakamoto, S. et al. Durable hematological response and improvement of nephrotic syndrome on thalidomide therapy in a patient with refractory light chain deposition disease. Int J Hematol 93, 673–676 (2011). https://doi.org/10.1007/s12185-011-0829-4
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12185-011-0829-4