Part 3: Diagnosis and Management of Immunoglobulin Light Chain Amyloidosis

Jason S. Starr, DO

Hematology/Oncology Fellow, Division of Hematology and Medical Oncology, Department of Medicine, Mayo Clinic, Jacksonville, FL

Taimur Sher, MD

Assistant Professor of Medicine, Division of Hematology and Medical Oncology, Department of Medicine, Mayo Clinic, Jacksonville, FL

Question 1

A 67-year-old Caucasian woman who never smoked presents with a 3-week history of hoarseness of voice. Laryngoscopy reveals decreased mobility of the right vocal cord due to a 0.5-cm submucosal nodule. Biopsy of the lesion demonstrates extracellular amorphous material staining positive with Congo red with apple green birefringence under polarized light. Immunohistochemistry and laser capture mass spectrometry examination of the congophilic material confirms immunoglobulin lambda light chain as the precursor protein. She has no signs or symptoms of systemic disease, and the blood work does not demonstrate any evidence of clonal plasma cell dyscrasia. What is the next step in management?

  •   Referral for endoscopic laser treatment
  •   Bone marrow biopsy
  •   Fat pad biopsy
  •   Initiation of melphalan and dexamethasone
  •   Refer for high-dose melphalan therapy and autologous stem cell rescue

Updated 8/09/2013 • jdw | Copyright ©2014 Turner White Communications