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High-dose melphalan followed by auto-SCT has favorable safety and efficacy in selected patients with light chain deposition disease and light and heavy chain deposition disease

Bone Marrow Transplantation volume 47, pages 453–455 (2012)Cite this article

Light chain and light and heavy chain deposition disease (LCDD and LHCDD) are plasma cell dyscrasias in which aggregation and deposition of Ig components leads to organ dysfunction. Although similar in principle to light chain (AL) amyloidosis, there are a number of distinguishing clinical and pathological features.1 Untreated, the natural history is usually progression to end-stage renal disease with death resulting either from light-chain-mediated organ impairment or complications of concomitant multiple myeloma (MM). Alkylator-based chemotherapy is generally ineffective in controlling disease progression, and high dose therapy with autologous stem cell transplant (ASCT) has been advocated as a means to effect a rapid reduction in pathological light chain production.2, 3 Data supporting the safety and efficacy of this approach remain limited given the rarity of these conditions.

We reviewed all eight patients with LCDD or LHCDD who underwent ASCT at our institution between August 2006 and November 2009. Baseline characteristics are listed in the Table 1. The median age of patients at presentation was 48 (40–62 years old). All patients presented with symptomatic renal disease including three with nephrotic syndrome, one with nephritic syndrome and one with acute renal insufficiency requiring dialysis. Renal biopsy demonstrated characteristic findings of light or light and heavy chain deposition in all patients and none had concurrent myeloma cast nephropathy or AL amyloidosis. Two patients met other criteria for symptomatic MM, such as the presence of otherwise unexplained anemia, hypercalcemia or lytic bone disease. No patients had evidence of extrarenal LCDD/LHCDD based on clinical examination, cardiac assessment by echocardiography or radionuclide angiography, pulmonary function testing or routine biochemistry.

Table 1 Presenting features, treatment and outcomes
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Authors and Affiliations

  1. Division of Hematology, Department of Medicine, University of British Columbia, Vancouver, British Columbia, Canada

    D Telio, J Shepherd, D Forrest, L Zypchen, M Barnett, T Nevill & K W Song

  2. The Leukemia/BMT Program of British Columbia, Vancouver General Hospital, Vancouver, British Columbia, Canada

    J Shepherd, D Forrest, M Barnett, T Nevill & K W Song

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  1. D Telio
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  2. J Shepherd
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Correspondence to K W Song.

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Telio, D., Shepherd, J., Forrest, D. et al. High-dose melphalan followed by auto-SCT has favorable safety and efficacy in selected patients with light chain deposition disease and light and heavy chain deposition disease. Bone Marrow Transplant 47, 453–455 (2012). https://doi.org/10.1038/bmt.2011.87

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