title: Heavy and Light chain amyloidosois presenting as complete heart block: A rare presentation of a rare disease.
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.
authors.affiliation: Department of Nephrology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India.
authors.forename: P S
authors.initials: PS
authors.lastname: Priyamvada
authors.affiliation: Department of Nephrology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India.
authors.forename: S
authors.initials: S
authors.lastname: Morkhandikar
authors.affiliation: Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India.
authors.forename: B H
authors.initials: BH
authors.lastname: Srinivas
authors.affiliation: Department of Nephrology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India.
authors.forename: S
authors.initials: S
authors.lastname: Parameswaran
keywords: Complete heart block; heavy and light chain amyloidosis; multiple myeloma; systemic amyloidosis
references.citation: Kidney Int. 2013 Mar;83(3):463-70
references.pmid: 23302715
references.citation: Circulation. 2005 Sep 27;112(13):2047-60
references.pmid: 16186440
references.citation: Arch Pathol Lab Med. 2007 Jun;131(6):917-22
references.pmid: 17550319
references.citation: Virchows Arch. 2005 Oct;447(4):756-61
references.pmid: 16021505
references.citation: Clin J Am Soc Nephrol. 2013 Sep;8(9):1515-23
references.pmid: 23704299
references.citation: Blood. 2009 Dec 3;114(24):4957-9
references.pmid: 19797517
references.citation: J Am Heart Assoc. 2012 Apr;1(2):e000364
references.pmid: 23130126