Open AccessHeavy and Light chain amyloidosois presenting as complete heart block: A rare presentation of a rare disease
Author(s) -
P S Priyamvada,
S Morkhandikar,
B H Srinivas,
Sreejith Parameswaran
Publication year - 2015
Publication title -
indian journal of nephrology/indian journal of nephrology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.317
H-Index - 24
eISSN - 1998-3662
pISSN - 0971-4065
DOI - 10.4103/0971-4065.135911
Subject(s) - immunoglobulin light chain , amyloidosis , medicine , al amyloidosis , rare disease , multiple myeloma , pathology , presentation (obstetrics) , amyloid (mycology) , antibody , disease , immunology , surgery
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.