Light-Chain Deposition Disease Treatment & Management

  • Author: Yasodah Jayamohan, MD; Chief Editor: Emmanuel C Besa, MD   more...
 
Updated: Dec 2, 2011
 

Medical Care

Treatment for LCDD should be aimed at reducing the clonal plasma cells responsible for the production of immunoglobulin light chains.

Treatment options for patients with LCDD include the following:

  • Cytotoxic chemotherapy, such as melphalan/prednisone, chlorambucil, or azathioprine[1, 9, 13, 26]
    • Intermediate-dose infusion regimens, including vincristine, doxorubicin, and dexamethasone (VAD) or vincristine, doxorubicin, and methylprednisolone (VAMP) may also be used. Newer therapies applicable to multiple myeloma such as lenolidomide and/or bortezomib may be considered, but data are limited.[31, 32]
  • Autologous stem cell transplantation
  • Kidney transplantation

Treatment for LCDD is variable. Although a few patients with renal disease with no evidence of myeloma are not treated, most receive chemotherapy as used for myeloma.[31] These patients show varying outcome (eg, one study showed a better outcome with vincristine-doxorubicin-dexamethasone/methylprednisolone therapy).

Studies demonstrating the role of autologous stem cell transplantation alone for patients with LCDD are very limited. Firkin et al reported a case of LCDD in which dialysis-dependent renal failure was reversed following autologous peripheral stem cell transplantation.[14]

Nonetheless, high-dose melphalan therapy with stem cell transplantation has been used in a small number of patients with LCDD, most of whom had concurrent myeloma. These studies demonstrate better outcome with minimal side effects.[1, 13]

A study by Royer et al included 11 patients, 10 of whom had multiple myeloma.[13] Following high-dose chemotherapy with stem cell transplantation, complete hematologic response was achieved in 6 patients, and a partial response in 2 patients. In addition, renal, cardiac, and hepatic functions improved in 6 patients, and regression of light-chain deposits in cardiac, liver, and skin biopsies were observed. Monoclonal immunoglobulin levels decreased in 8 patients, with complete disappearance from urine and serum in 6 cases. No deaths related to treatment were observed.[13]

A similar experience was observed by Weichman et al who treated 6 cases of LCDD with high-dose melphalan and autologous stem cell transplantation.[1] However, no history of myeloma was noted. A complete hematologic response was achieved in 5 of 6 patients, and all were alive at a median follow-up of 12 months.[1] Moreover, the serum free light-chain levels normalized in all 6 patients. Again, there were no treatment-related deaths.

The above studies show that high-dose melphalan with subsequent autologous stem cell transplantation may be the treatment of choice for patients with LCDD regardless of their myeloma status. The hematologic responses were acceptable with regression of light-chain deposits and low treatment-related mortality rates.

Kidney transplantation has been performed in small number of patients with LCDD. The disease recurs in the allograft in 80% of cases, and the outcome is poor due to recurrence of disease in the renal allograft or progression of the underlying plasma cell disorder.

In a study by Leung et al, 7 patients with LCDD who were treated with kidney transplantation were retrospectively reviewed.[33] All 7 had nephrotic-range proteinuria and were maintained on hemodialysis before transplantation. LCDD recurred in 5 of 7 patients. One patient died of progression of multiple myeloma without evidence of recurrence. One patient remained free of disease 13 years after the kidney transplantation.[33] The authors concluded that renal transplantation should not be considered as a treatment option unless measures have been taken to reduce light-chain production.

The potential role rituximab is questionable. A case was reported in which therapy with rituximab delayed the recurrence of LCDD in a patient with second renal allograft.[34]

Proceed to Medication
 
 
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 Association for the Advancement of Science, American Association of Blood Banks, American Clinical and Climatological Association, American Society for Clinical Pathology, American Society of Hematology, College of American Pathologists, International Society of Blood Transfusion, International Society on Thrombosis and Haemostasis, and Royal College of Physicians and Surgeons of Canada

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.

Specialty Editor Board

Paul Schick, MD  Emeritus Professor, Department of Internal Medicine, Jefferson Medical College of Thomas Jefferson University; Research Professor, Department of Internal Medicine, Drexel University College of Medicine; Adjunct Professor of Medicine, Lankenau Hospital

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.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

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.

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, Jefferson Medical College of 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 Clinical Oncology, American Society of Hematology, and New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

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

References

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