eMedicine Specialties > Hematology > Plasma Cell Disorders

Light-Chain Deposition Disease: Follow-up

Author: Yasodah Jayamohan, MD, Transfusion Medicine Fellow, Hoxworth Blood Center, University of Cincinnati Medical Center
Coauthor(s): Ronald A Sacher, MB, BCh, MD, FRCPC, Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center; Suzanne R Fanning, DO, Fellow, Department of Hematology and Medical Oncology, Cleveland Clinic Foundation, 2004-2007 Director, Hematology, Greenville Memorial Health System, Greenville, SC Medical Oncologist/Hematologist/Transplant Physician, Cancer Centers of the Carolinas; Mohamad A Hussein, MD, Clinical Director, Malignant Hematology, Moffitt Cancer Center
Contributor Information and Disclosures

Updated: Jun 4, 2008

Follow-up

Prognosis

  • Patient and renal survival in patients with LCDD is variable, ranging from 1 month to 10 years. If untreated, ESRD occurs in 70% of cases. A study by Pozzi et al which included 63 patients demonstrated 52% patient and 40% renal survival rates for a 4-year period.32 Renal prognosis is affected by the patient's age and serum creatinine levels at presentation.
  • Overall survival has been shown to be influenced by patient age, the presence of coexisting multiple myeloma or cast nephropathy, and any evidence of extrarenal light-chain deposition.   
  • The natural or treated history of LCDD is difficult to determine as this disease is uncommon. 

Miscellaneous

Medicolegal Pitfalls

  • Failure to diagnose LCDD may lead to the inability to prevent or delay the progression of end-organ damage and/or allow for screening for transformation to a neoplastic process. Note: This statement should be cautiously interpreted because the disease does not present with common signs or symptoms, and the impact of therapy on the outcome is not well studied.
 
Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous coauthor Dr Jaya Juturi to the development and writing of this article.



More on Light-Chain Deposition Disease

Overview: Light-Chain Deposition Disease
Differential Diagnoses & Workup: Light-Chain Deposition Disease
Treatment & Medication: Light-Chain Deposition Disease
Follow-up: Light-Chain Deposition Disease
References
Further Reading
[ CLOSE WINDOW ]

References

  1. Weichman K, Dember LM, Prokaeva T, 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. Sep 2006;38(5):339-43. [Medline].

  2. 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. Nov 2006;1(6):1342-50. [Medline]. [Full Text].

  3. Buxbaum JN, Chuba JV, Hellman GC, Solomon A, Gallo GR. Monoclonal immunoglobulin deposition disease: light chain and light and heavy chain deposition diseases and their relation to light chain amyloidosis. Clinical features, immunopathology, and molecular analysis. Ann Intern Med. Mar 15 1990;112(6):455-64. [Medline].

  4. Randall RE, Williamson WC Jr, Mullinax F, Tung MY, Still WJ. Manifestations of systemic light chain deposition. Am J Med. Feb 1976;60(2):293-9. [Medline].

  5. Tubbs RR, Gephardt GN, McMahon JT, et al. Light chain nephropathy. Am J Med. Aug 1981;71(2):263-9. [Medline].

  6. Seymour AE, Thompson AJ, Smith PS, Woodroffe AJ, Clarkson AR. Kappa light chain glomerulosclerosis in multiple myeloma. Am J Pathol. Dec 1980;101(3):557-80. [Medline]. [Full Text].

  7. Alpers CE, Tu WH, Hopper J Jr, Biava CG. Single light chain subclass (kappa chain) immunoglobulin deposition in glomerulonephritis. Hum Pathol. Mar 1985;16(3):294-304. [Medline].

  8. Brockhurst I, Harris KP, Chapman CS. Diagnosis and monitoring a case of light-chain deposition disease in the kidney using a new, sensitive immunoassay. Nephrol Dial Transplant. Jun 2005;20(6):1251-3. [Medline]. [Full Text].

  9. 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. Jul 1992;20(1):34-41. [Medline].

  10. Confalonieri R, Barbiano di Belgiojoso G, et al. Light chain nephropathy: histological and clinical aspects in 15 cases. Nephrol Dial Transplant. 1988;3(2):150-6. [Medline].

  11. Hill GS, Morel-Maroger L, Méry JP, Brouet JC, Mignon F. Renal lesions in multiple myeloma: their relationship to associated protein abnormalities. Am J Kidney Dis. Jan 1983;2(4):423-38. [Medline].

  12. Lorenz EC, Gertz MA, Fervenza FC, et al. Long-term outcome of autologous stem cell transplantation in light chain deposition disease. Nephrol Dial Transplant. Jun 2008;23(6):2052-7. [Medline].

  13. Royer B, Arnulf B, Martinez F, et al. High dose chemotherapy in light chain or light and heavy chain deposition disease. Kidney Int. Feb 2004;65(2):642-8. [Medline].

  14. 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. Sep 2004;44(3):551-5. [Medline]. [Full Text].

  15. Rongioletti F, Patterson JW, Rebora A. The histological and pathogenetic spectrum of cutaneous disease in monoclonal gammopathies. J Cutan Pathol. Mar 10 2008;epub ahead of print. [Medline].

  16. Popovic M, Tavcar R, Glavac D, Volavsek M, Pirtosek Z, Vizjak A. Light chain deposition disease restricted to the brain: The first case report. Hum Pathol. Jan 2007;38(1):179-84. [Medline].

  17. Ganeval D, Noël LH, Preud'homme JL, Droz D, Grünfeld JP. Light-chain deposition disease: its relation with AL-type amyloidosis. Kidney Int. Jul 1984;26(1):1-9. [Medline].

  18. Bradley JR, Thiru S, Evans DB. Light chains and the kidney. J Clin Pathol. Jan 1987;40(1):53-60. [Medline]. [Full Text].

  19. Gallo GR, Feiner HD, Katz LA, et al. Nodular glomerulopathy associated with nonamyloidotic kappa light chain deposits and excess immunoglobulin light chain synthesis. Am J Pathol. Jun 1980;99(3):621-44. [Medline].

  20. Noel LH, Droz D, Ganeval D, Grunfeld JP. Renal granular monoclonal light chain deposits: morphological aspects in 11 cases. Clin Nephrol. May 1984;21(5):263-9. [Medline].

  21. Keeling J, Herrera GA. Matrix metalloproteinases and mesangial remodeling in light chain-related glomerular damage. Kidney Int. Oct 2005;68(4):1590-603. [Medline].

  22. Bhargava P, Rushin JM, Rusnock EJ, et al. Pulmonary light chain deposition disease: report of five cases and review of the literature. Am J Surg Pathol. Feb 2007;31(2):267-76. [Medline].

  23. Colombat M, Mal H, Copie-Bergman C, et al. Primary cystic lung light chain deposition disease: a clinicopathologic entity derived from unmutated B cells with a stereotyped IGHV4-34/IGKV1 receptor. Blood. May 15 2008;epub ahead of print. [Medline].

  24. Mallick NP, Dosa S, Acheson EJ, Delamore IW, et al. Detection, significance and treatment of paraprotein in patients presenting with 'idiopathic' proteinuria without myeloma. Q J Med. Apr 1978;47(186):145-75. [Medline].

  25. Pirani CL, Silva F, D'Agati V, Chander P, Striker LM. Renal lesions in plasma cell dyscrasias: ultrastructural observations. Am J Kidney Dis. Sep 1987;10(3):208-21. [Medline].

  26. Gipstein RM, Cohen AH, Adams DA, Adams T, Grabie MT. Kappa light chain nephropathy without evidence of myeloma cells. Response to chemotherapy with cessation of maintenance hemodialysis. Am J Nephrol. 1982;2(5):276-81. [Medline].

  27. Kaplan B, Ramirez-Alvarado M, Dispenzieri A, et al. Isolation and biochemical characterization of plasma monoclonal free light chains in amyloidosis and multiple myeloma: a pilot study of intact and truncated forms of light chains and their charge properties. Clin Chem Lab Med. 2008;46(3):335-41. [Medline]. [Full Text].

  28. Knobler H, Kopolovic J, Kleinman Y, et al. Multiple myeloma presenting as dense deposit disease. Light chain nephropathy. Nephron. 1983;34(1):58-63. [Medline].

  29. San-Miguel J, Harousseau JL, Joshua D, Anderson KC. Individualizing treatment of patients with myeloma in the era of novel agents. J Clin Oncol. Jun 1 2008;26(16):2761-6. [Medline].

  30. Leung N, Lager DJ, Gertz MA, et al. Long-term outcome of renal transplantation in light-chain deposition disease. Am J Kidney Dis. Jan 2004;43(1):147-53. [Medline].

  31. Kuypers DR, Lerut E, Claes K, Evenepoel P, Vanrenterghem Y. Recurrence of light chain deposit disease after renal allograft transplantation: potential role of rituximab?. Transpl Int. Apr 2007;20(4):381-5. [Medline].

  32. Pozzi C, D'Amico M, Fogazzi GB, et al. Light chain deposition disease with renal involvement: clinical characteristics and prognostic factors. Am J Kidney Dis. Dec 2003;42(6):1154-63. [Medline].

  33. Kaplan EL, Meier P. Nonparametric estimation from incomplete observations. J Am Stat Assoc. 1958;53:457-81.

  34. Kyle RA. Multiple myeloma and the dysproteinemias. In: Stein JH, ed. Internal Medicine. 3rd ed. Boston, Mass: Little Brown & Co; 1990:1146-52.

  35. Kyle RA, Garton JP. Laboratory monitoring of myeloma proteins. Semin Oncol. Sep 1986;13(3):310-7. [Medline].

  36. Sikkink LA, Ramirez-Alvarado M. Biochemical and aggregation analysis of Bence Jones proteins from different light chain diseases. Amyloid. Mar 2008;15(1):29-39. [Medline]. [Full Text].

  37. Tanenbaum ND, Howell DN, Middleton JP, Spurney RF. Lambda light chain deposition disease in a renal allograft. Transplant Proc. Dec 2005;37(10):4289-92. [Medline].

  38. Velosa JA, Holley KE. Pathology and immunopathology of renal diseases. In: Duarte CG, ed. Renal Function Tests: Clinical Laboratory Procedures and Diagnosis. Boston, Mass: Little Brown & Co; 1980:347-85.

[ CLOSE WINDOW ]

Keywords

LCDD, light chain deposition disease, light-chain disease, light chain disease, free light chains, FLC, renal disease, renal insufficiency, proteinuria, nephrotic syndrome, multiple myeloma, lymphoproliferative disease, lymphoproliferative disorder, end-stage renal disease, ESRD, monoclonal gammopathies, gammopathies, monoclonal gammopathy of unknown significance, MGUS, Bence Jones protein, BJP, immunoglobulin-related amyloidosis, AL-amyloidosis

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Yasodah Jayamohan, MD, Transfusion Medicine Fellow, Hoxworth Blood Center, University of Cincinnati Medical Center
Yasodah Jayamohan, MD is a member of the following medical societies: American Society for Clinical Pathology, American Society of Cytopathology, College of American Pathologists, and United States and Canadian Academy of Pathology
Disclosure: Nothing to disclose.

Coauthor(s)

Ronald A Sacher, MB, BCh, MD, FRCPC, Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center
Ronald A Sacher, MB, BCh, MD, FRCPC is a member of the following medical societies: American Society of Hematology
Disclosure: Glaxo Smith Kline Honoraria Speaking and teaching; Talecris Honoraria Board membership

Suzanne R Fanning, DO, Fellow, Department of Hematology and Medical Oncology, Cleveland Clinic Foundation, 2004-2007 Director, Hematology, Greenville Memorial Health System, Greenville, SC Medical Oncologist/Hematologist/Transplant Physician, Cancer Centers of the Carolinas
Suzanne R Fanning, DO is a member of the following medical societies: American College of Physicians, American Medical Association, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, and American Society of Hematology
Disclosure: Millenium Pharmaceuticals Consulting fee Review panel membership; Celgene Pharmaceuticals Consulting fee Review panel membership

Mohamad A Hussein, MD, Clinical Director, Malignant Hematology, Moffitt Cancer Center
Mohamad A Hussein, MD is a member of the following medical societies: American Association of Blood Banks, American College of Physicians, American Medical Association, and American Society of Hematology
Disclosure: Nothing to disclose.

Medical Editor

Paul Schick, MD, Emeritus Professor, Department of Internal Medicine, Thomas Jefferson University Medical College; Research Professor, Department of Internal Medicine, Drexel University College of Medicine; Adjunct Professor of Medicine, Lankenau Hospital, Wynnewood, PA
Paul Schick, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Society of Hematology, International Society on Thrombosis and Haemostasis, and New York Academy of Sciences
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Troy H Guthrie, Jr, MD, Director of Cancer Institute, Baptist Medical Center
Troy H Guthrie, Jr, MD is a member of the following medical societies: American Federation for Medical Research, American Medical Association, American Society of Hematology, Florida Medical Association, Medical Association of Georgia, and Southern Medical Association
Disclosure: Nothing to disclose.

CME Editor

Rajalaxmi McKenna, MD, FACP, Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Thomas Jefferson University
Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.