Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Letter to the Editor
  • Published:

Long-term outcome of patients with monoclonal Ig deposition disease treated with high-dose melphalan and stem cell transplantation

Bone Marrow Transplantation volume 46, pages 161–162 (2011)Cite this article

Subjects

Monoclonal Ig deposition disease (MIDD) is a clonal plasma cell dyscrasia in which light-chain and/or heavy-chain subunits of Igs form non-fibrillar deposits in various tissues, causing organ dysfunction.1 Kidneys are universally affected, often presenting with proteinuria and rapidly progressing to end-stage renal failure in more than 60% of cases.1 Extra-renal manifestations are less common, but can include restrictive cardiomyopathy, myocardial infarction from coronary deposition of Igs, cholestatic jaundice, hepatic failure, cerebral infarction and hemorrhage, adrenal insufficiency, peripheral neuropathy, mononeuritis multiplex, and soft tissue deposition.1, 2 High-dose melphalan (HDM) and autologous SCT (auto-SCT) has a role in the treatment of MIDD.3, 4 We previously reported on a case series in which five of the six patients (83%) treated with HDM/auto-SCT achieved a complete hematological response without any treatment-related mortality or significant morbidity.3 This case series was updated in 2007, and a complete hematological response was achieved in seven of the eight patients (88%) with a median follow-up of 17 months (range, 4–41).1 Here, we report on a long-term hematological and clinical follow-up in patients with MIDD treated with HDM/auto-SCT.

Between 2003 and 2008, 10 patients with MIDD were treated with HDM/auto-SCT at the Boston University Medical Center. Their median age was 45 years (range, 34–70) and nine of the patients were male. Six patients had kappa clonal plasma cell dyscrasia and four patients had lambda clonal plasma cell dyscrasia. The median BM plasma cell percentage before HDM/auto-SCT was 10% (range, 5–30%). Four patients had received treatment previously: one had received bortezomib, one high-dose dexamethasone, one oral melphalan and prednisone, and one thalidomide and dexamethasone. All patients had kidney involvement, two required treatment with chronic dialysis and one had undergone kidney transplantation. Two patients (20%) had cardiac involvement. Among the non-dialysis-dependent patients (n=8), the median serum creatinine level was 3.1 mg/dL (range, 1.1–4.9) and the median creatinine clearance was 28.5 mL/min (range, 19–128). The median 24-h urine total protein excretion for the whole group of patients was 4037 mg (range, 458–15 632).

This is a preview of subscription content, access via your institution

Access options

Buy this article

  • Purchase on Springer Link
  • Instant access to full article PDF
Buy now

Prices may be subject to local taxes which are calculated during checkout

Figure 1

References

  1. Salant DJ, Sanchorawala V, D’Agati VD . A case of atypical light chain deposition disease–diagnosis and treatment. Clin J Am Soc Nephrol 2007; 2: 858–867.

    Article  Google Scholar 

  2. 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: 1154–1163.

    Article  Google Scholar 

  3. 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: 339–343.

    Article  CAS  Google Scholar 

  4. 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: 642–648.

    Article  CAS  Google Scholar 

  5. 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: 300–302.

    Article  CAS  Google Scholar 

  6. 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: 2052–2057.

    Article  CAS  Google Scholar 

  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: 405–412.

    Article  CAS  Google Scholar 

  8. Skinner M, Sanchorawala V, Seldin DC, Dember LM, Falk RH, Berk JL et al. High-dose melphalan and autologous stem-cell transplantation in patients with AL amyloidosis: an 8-year study. Ann Intern Med 2004; 140: 85–93.

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Amyloid Treatment and Research Program, Boston University School of Medicine, Boston, MA, USA

    S Girnius, D C Seldin, L M Dember, A Segal & V Sanchorawala

  2. Stem Cell Transplantation Program of the Section of Hematology-Oncology, Boston University School of Medicine, Boston, MA, USA

    D C Seldin, K Quillen & V Sanchorawala

  3. Department of Pathology and Laboratory Medicine, Boston University School of Medicine, Boston, MA, USA

    K Quillen

  4. Renal Section, Boston University School of Medicine, Boston, MA, USA

    L M Dember & A Segal

Authors
  1. S Girnius
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. D C Seldin
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. K Quillen
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. L M Dember
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. A Segal
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. V Sanchorawala
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to V Sanchorawala.

Ethics declarations

Competing interests

The authors declare no conflict of interest.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Girnius, S., Seldin, D., Quillen, K. et al. Long-term outcome of patients with monoclonal Ig deposition disease treated with high-dose melphalan and stem cell transplantation. Bone Marrow Transplant 46, 161–162 (2011). https://doi.org/10.1038/bmt.2010.82

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1038/bmt.2010.82

Search

Advanced search

Quick links