A Case of Monoclonal Immunoglobulin Deposition Disease – on Bortezomib regimen, A Novel strategy in Multisystemic Involvement.
Rupesh S. Parati*
, Ponvijaya M. Yadav, Vijayshree S. Gokhale and Atiullah Imran MalikDepartment of Medicine, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Dr. D. Y. Patil Vidyapeeth, Pune, India.
Corresponding Author E-mail:rupeshparati@gmail.com
Abstract: Background: Monoclonal immunoglobulin deposition disease (MIDD) is a rare and sporadic phenomenon often manifesting in individuals who are in their 5th – 6th decade of life. MIDD along with restrictive cardiomyopathy and Renal AL amyloidosis as in the present case is also an unaccustomed phenomenon. Often, the patient presents with unusual symptoms and is more prone to misdiagnosis. The congo red (-) deposition of the monoclonal light chain can be frequently noted in multiple organs including the heart and kidney. The light chain deposition can be either isolated lambda or gamma chain and they are classified accordingly. While serum electrophoresis at an early presentation may reveal underlying monoclonal gammopathy, the immunofluorescence and histopathological examination of renal biopsy remain the mainstay diagnostic tool. Objective: The case study was done because of its uniqueness in the usage of a novel strategy in the treatment of MIDD. Results: In the current case, a 52-year-old male presented with chest pain and NYHA grade 3 dyspnea for 8 days along with bilateral lower limb pitting oedema since 3 months. In certain prevailing situations, a patient can be misdiagnosed with ischemic heart disease or acute on chronic renal failure. However, detailed history and time-bound investigation will act as a safeguard. Conclusion: When patients have no co-morbidities and no addictions, and the patient’s investigation reveals heavy proteinuria, the rheumatological and haematological etiologies must be ruled out to establish a final diagnosis for timely treatment. As in our case, the rheumatological work-up was negative but we found an M band spike on serum electrophoresis, which expedited our search for underlying plasma-cell dyscrasia.
Keywords: AL Renal Amyloidosis; Bortezomib; Dexamethasone; Monoclonal Immunoglobulin Deposition Disease; Restrictive Cardiomyopathy Back to TOC