Primary systemic amyloidosis: a case report

Authors

  • Jayashankar CA Associate Professor, Department of General Medicine, Vydehi Institute of Medical Sciences and Research Centre, Bangalore - 560066, Karnataka
  • D. S. Somasekar Professor and Head, Department of General Medicine, Vydehi Institute of Medical Sciences and Research Centre, Bangalore - 560066, Karnataka
  • Pavan Kumar Perugu Post graduate, Department of General Medicine, Vydehi Institute of Medical Sciences and Research Centre, Bangalore - 560066, Karnataka
  • Santosh KV Professor, Department of Pathology, Vydehi Institute of Medical Sciences and Research Centre, Bangalore - 560066, Karnataka
  • Manjunath Nandennavar Assistant Professor, Department of Medical Oncology, Vydehi Institute of Medical Sciences and Research Centre, Bangalore - 560066, Karnataka
  • Praveen Mathew Associate Professor, Department of Medical Gastroenterology, Vydehi Institute of Medical Sciences and Research Centre, Bangalore - 560066, Karnataka

Keywords:

AL Amyloidosis, Proteinuria, Hepatomegaly

Abstract

Primary amyloidosis is a group of monoclonal plasma cell disorders, characterized by extracellular deposition of immunoglobulin light chain fibrils in multiple organs leading to progressive multiorgan dysfunction. It is a rare disease which usually occurs in elderly persons and has a poor prognosis. We report a 72-year-old male patient with chronic abdominal distension, bilateral pitting pedal edema with nephrotic range proteinuria and amyloid deposition in liver and bone marrow. Immunoelectrophoresis of serum demonstrated the presence of immunoglobulin light chains of the circulating monoclonal protein. He was treated with IV bortezomib and IV dexamethasone.

Metrics

Metrics Loading ...

References

Harsh Mohan. Immunopathology including amyloidosis. In: Praveen Mohan, Tanya Mohan, Sugandha Mohan, eds. Text Book of Pathology. 5th ed. New Delhi: Jaypee brothers; 2005: 83-92.

Khan MF, Falk RH. Amyloidosis. Postgrad Med J. 2001 Nov;77(913):686-93.

Estelle Desport, Frank Bridoux, Christophe Sirac, Sebastien Delbes, Sebastien Bender, Beatrice Fernandez et al. AL Amyloidosis. Orphan J Rare Dis. 2012 Aug;7:54.

Saoji V, Chaudhari S, Gohokar D. Primary systemic amyloidosis: three different presentations. Indian J Dermatol Venerol Leprol. 2009 Jul-Aug;75(4):394-7.

Rajkumar SV, Dispenzieri A, Kyle RA. Monoclonal gammopathy of undetermined significance, waldenstrom macroglobulinemia, AL amyloidosis, and related plasma cell disorders: diagnosis and treatment. Mayo Clin Proc. 2006;81:693-703.

Gertz MA, Comenzo R, Falk RH, Fermand JP, Hazenberg BP, Hawkins PN et al. Definition of organ involvement and treatment response in immunoglobulin light chain amyloidosis (AL): a consensus opinion from the 10th international symposium on amyloid and amyloidosis, tours, France, 18-22 April 2004. Am J Hematol. 2005;79:319-328.

Gertz MA, Merlini G. Definition of organ involvement and response to treatment in AL amyloidosis: an updated consensus opinion. Amyloid. 2010;17(Suppl 1):48-9.

Downloads

Published

2017-01-23

How to Cite

CA, J., Somasekar, D. S., Perugu, P. K., KV, S., Nandennavar, M., & Mathew, P. (2017). Primary systemic amyloidosis: a case report. International Journal of Research in Medical Sciences, 2(2), 744–748. Retrieved from https://www.msjonline.org/index.php/ijrms/article/view/2234

Issue

Vol. 2 No. 2 (2014): April-June 2014

Section

Case Reports