Clinical, Laboratory Profile, Treatment and Outcome Of Primary Amyloidosis in the Era Of Novel Agents: An Experience from a Tertiary Care Hospital

ABSTRACT

Introduction: Diagnosis of AL amyloidosis requires demonstration of amyloid in affected tissues along with clonal plasma cells in bone marrow or presence of monoclonal light chains in blood. With the availability of serum light chain assay and immunophenotyping by flow cytometry, more cases of AL amyloidosis are being diagnosed. Here we present our experience of AL amyloidosis diagnosis and treatment in the era of modern diagnostics and therapy. Aims & Objectives: We aimed to describe the clinical presentations, laboratory features, treatment and outcomes of patients with AL amyloidosis in a single center using standard diagnostic tests and treatment with novel agents. Material(s) and Method(s) A retrospective analysis of AL amyloidosis patients, diagnosed in our hospital, a tertiary care center from January 2016 to June 2022 was conducted. The data was collected from departmental database. All statistical analyses were done by SPSS version 17. Result(s) Diagnosis of AL amyloidosis was done in 31 patients. Median age of presentation was 61 years. 25 (80.6%) were males. Major symptoms were pedal edema (38.7%) and shortness of breath (32.3%). Twenty four (77.4%) presented with ECOG PS >= 2. Most common systems involved were cardiac (54.8%) and renal (54.8%). Fourteen (45.2%) had two or more systems involvement while 17 (54.8%) had single system involvement. Lambda monoclonal light chain was present in 83.9% and kappa monoclonal light chain in 16.1%. Median M-protein was 0.59 g/dL (range 0-2 g/dL) and median bone marrow plasma cells were 6% (range-1-18%). Eighteen patients were treated;cyclophosphamide, bortezomib and dexamethasone (CyBORD) in 12/18 (66.7%) and bortezomib + dexamethasone in 6/18 (33.3%). Among 18 patients followed up with median follow up of 9 months (range 1-64 months), six expired;three due to COVID, two due to cardiac arrhythmia (during first cycle) and one due to relapse and rest 12 were alive. Among the 12 patients who were alive 6 were in complete hematological response. Conclusion(s) Our study presents the spectrum of clinical manifestations, management and outcomes of primary amyloidosis in Indian context. There is a need to increase the awareness among the physicians about amyloidosis so that early diagnosis can be made and timely treatment can be done with novel agents to improve the dismal historical results.