Heavy and Light chain amyloidosois presenting as complete heart block: A rare presentation of a rare disease

P S Priyamvada; S Morkhandikar; B H Srinivas; S Parameswaran

doi:10.4103/0971-4065.135911

Heavy and Light chain amyloidosois presenting as complete heart block: A rare presentation of a rare disease

Indian J Nephrol. 2015 Mar-Apr;25(2):106-9. doi: 10.4103/0971-4065.135911.

Authors

P S Priyamvada¹, S Morkhandikar¹, B H Srinivas², S Parameswaran¹

Affiliations

¹ Department of Nephrology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India.
² Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India.

Abstract

Amyloidosis is an uncommon disease characterized by deposition of proteinaceous material in the extracellular matrix, which results from abnormal protein folding. Even though more than 25 precursor proteins are identified, majority of systemic amyloidosis results from deposition of abnormal immunoglobulin (Ig) light chains. In heavy chain amyloidosis (AH), deposits are derived from both heavy chain alone, whereas in heavy and light chain amyloidosis (AHL), the deposits are derived from Ig heavy chains and light chains. Both AH and AHL are extremely rare diseases. Here, we report an unusual presentation of IgG (lambda) AHL amyloidosis in the background of multiple myeloma, where the initial clinical presentation was complete heart block, which preceded the definitive diagnosis by 18 months.

Keywords: Complete heart block; heavy and light chain amyloidosis; multiple myeloma; systemic amyloidosis.

Publication types

Case Reports