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Chronic myopathy due to immunoglobulin light chain amyloidosis.

Publication ,  Journal Article
Manoli, I; Kwan, JY; Wang, Q; Rushing, EJ; Tsokos, M; Arai, AE; Burch, WM; Dispenzieri, A; McPherron, AC; Gahl, WA
Published in: Mol Genet Metab
April 2013

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.

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Published In

Mol Genet Metab

DOI

EISSN

1096-7206

Publication Date

April 2013

Volume

108

Issue

4

Start / End Page

249 / 254

Location

United States

Related Subject Headings

  • Transplantation, Autologous
  • Satellite Cells, Skeletal Muscle
  • Paraproteinemias
  • Muscular Diseases
  • Immunoglobulin Light Chains
  • Humans
  • Genetics & Heredity
  • Female
  • Bone Marrow Transplantation
  • Amyloidosis
 

Citation

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Manoli, I., Kwan, J. Y., Wang, Q., Rushing, E. J., Tsokos, M., Arai, A. E., … Gahl, W. A. (2013). Chronic myopathy due to immunoglobulin light chain amyloidosis. Mol Genet Metab, 108(4), 249–254. https://doi.org/10.1016/j.ymgme.2013.01.015
Manoli, Irini, Justin Y. Kwan, Qian Wang, Elisabeth J. Rushing, Maria Tsokos, Andrew E. Arai, Warner M. Burch, Angela Dispenzieri, Alexandra C. McPherron, and William A. Gahl. “Chronic myopathy due to immunoglobulin light chain amyloidosis.Mol Genet Metab 108, no. 4 (April 2013): 249–54. https://doi.org/10.1016/j.ymgme.2013.01.015.
Manoli I, Kwan JY, Wang Q, Rushing EJ, Tsokos M, Arai AE, et al. Chronic myopathy due to immunoglobulin light chain amyloidosis. Mol Genet Metab. 2013 Apr;108(4):249–54.
Manoli, Irini, et al. “Chronic myopathy due to immunoglobulin light chain amyloidosis.Mol Genet Metab, vol. 108, no. 4, Apr. 2013, pp. 249–54. Pubmed, doi:10.1016/j.ymgme.2013.01.015.
Manoli I, Kwan JY, Wang Q, Rushing EJ, Tsokos M, Arai AE, Burch WM, Dispenzieri A, McPherron AC, Gahl WA. Chronic myopathy due to immunoglobulin light chain amyloidosis. Mol Genet Metab. 2013 Apr;108(4):249–254.
Journal cover image

Published In

Mol Genet Metab

DOI

EISSN

1096-7206

Publication Date

April 2013

Volume

108

Issue

4

Start / End Page

249 / 254

Location

United States

Related Subject Headings

  • Transplantation, Autologous
  • Satellite Cells, Skeletal Muscle
  • Paraproteinemias
  • Muscular Diseases
  • Immunoglobulin Light Chains
  • Humans
  • Genetics & Heredity
  • Female
  • Bone Marrow Transplantation
  • Amyloidosis