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Scholars Journal of Medical Case Reports | Volume-7 | Issue-10
Ventricular Tachycardia as an Initial Manifestation of Randall-Type Monoclonal Immunoglobulin Deposition Disease: A Case Report and Literature
B. Maatof, M.Raouah, D.Benzeroual, M. El Hattaoui
Published: Oct. 30, 2019 | 136 143
DOI: 10.36347/SJMCR.2019.v07i10.012
Pages: 631-634
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Abstract
Monoclonal immunoglobulin deposition disease (MIDD) is a rare complication of B-cell clonal disorders, defined by Congo-red negative deposits of monoclonal light chain (LCDD), heavy chain (HCDD), or both (LHCDD). MIDD is a systemic disorder with prominent renal involvement but little attention has been paid to the description of extra-renal manifestations. Moreover, mechanisms of pathogenic immunoglobulin deposition and factors associated with renal and patient survival are ill-defined. Extra-renal involvement (most commonly cardiac) is seen in up to 10% of cases [1]. The frequency of cardiac manifestations is increasing with the duration of evolution of the disease. They they manifest themselves by restrictive hypertrophic cardiomyopathy, close to the amyloid heart disease, with disturbances in rhythm or conduction, associated with electrocardiographic micro voltage, and subsequently heart failure congestive, sometimes revealing the disease [1]. Our case describes an unusual presentation of Randall-type monoclonal immunoglobulin deposition revealed by rhythm disorder (VT).