Chronic myopathy due to immunoglobulin light chain amyloidosis.

Published

Journal Article

Amyloid myopathy associated with a plasma cell dyscrasia is a rare cause of muscle hypertrophy. It can be a challenging diagnosis, since pathological findings are often elusive. In addition, the mechanism by which immunoglobulin light-chain deposition stimulates muscle overgrowth remains poorly understood. We present a 53-year old female with a 10-year history of progressive generalized muscle overgrowth. Congo-red staining and immunohistochemistry revealed perivascular lambda light chain amyloid deposits, apparent only in a second muscle biopsy. The numbers of central nuclei and satellite cells were increased, suggesting enhanced muscle progenitor cell formation. Despite the chronicity of the light chain disease, the patient showed complete resolution of hematologic findings and significant improvement of her muscle symptoms following autologous bone marrow transplantation. This case highlights the importance of early diagnosis and therapy for this treatable cause of a chronic myopathy with muscle hypertrophy.

Full Text

Duke Authors

Cited Authors

  • Manoli, I; Kwan, JY; Wang, Q; Rushing, EJ; Tsokos, M; Arai, AE; Burch, WM; Dispenzieri, A; McPherron, AC; Gahl, WA

Published Date

  • April 2013

Published In

Volume / Issue

  • 108 / 4

Start / End Page

  • 249 - 254

PubMed ID

  • 23465863

Pubmed Central ID

  • 23465863

Electronic International Standard Serial Number (EISSN)

  • 1096-7206

International Standard Serial Number (ISSN)

  • 1096-7192

Digital Object Identifier (DOI)

  • 10.1016/j.ymgme.2013.01.015

Language

  • eng