How is light-chain deposition disease (LCDD) treated?

Updated: Sep 26, 2019
  • Author: Swapna Boppana, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Treatment of light-chain deposition disease (LCDD) is indicated for patients who present with systemic involvement, renal dysfunction, and associated presence of multiple myeloma. The goal of treatment in these patients is to suppress the production of light chains and damage to other organs. Appropriate medical management must be provided for organ dysfunction as needed, such as the use of angiotensin-converting enzyme (ACE) inhibitors or dialysis.

Unlike in multiple myeloma, the plasma cell burden is quite low (< 5%) and the genetic abnormalities associated with adverse prognosis in multiple myeloma are absent. In patients with LCDD associated with multiple myeloma, the prognosis is quite poor and they should be treated per multiple myeloma guidelines. [38] In these patients, there is no evidence to support maintenance therapy except for an anecdotal report in 1 patient who received thalidomide maintenance following chemotherapy. [39] LCDD is a rare disease, hence there are no established guidelines and management remains controversial. [14]

Treatment options include the following [40] :

  • Autologous stem cell transplantation (ASCT)

  • Bortezomib

  • Immunomodulatory drugs

  • Renal transplantation


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