What is the role of immunomodulatory drugs in the treatment of light-chain deposition disease (LCDD)?

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

Thalidomide is an immunomodulatory drug that has been extensively studied in amyloid light-chain (AL)–amyloidosis and multiple myeloma. The use in LCDD has been limited. A report in one young patient in whom conventional chemotherapy failed demonstrated that thalidomide with dexamethasone was able to provide a complete hematological response after 8 months. The patient had a sustained hematological response with improvement in renal function that lasted 31 months. [50]

Another case was reported in a patient with liver involvement who was given lenalidomide with melphalan and prednisone. However, lenalidomide was discontinued as the patient unfortunately developed intrahepatic ischemic cholangitis. [51]  

In another case, melphalan and prednisolone (MP) therapy was started upon diagnosis and continued for 10 years with serial evaluations of renal histology revealing the resolution of nodular lesions, and the glomeruli became nearly normal. [52] The immunomodulatory agents are promising but their role needs to be further explored in prospective studies.


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