Intricacies in the Diagnosis and Management of Al Amyloidosis: Experience from a Tertiary Care Institute in North India

Abstract

Aims: Primary (AL) amyloidosis is a form of systemic amyloidosis, causing organ dysfunction, mainly affecting the heart and kidney. Early recognition and diagnosis is critical in AL amyloidosis management. This study aimed to describe the clinical and laboratory profile and treatment pattern and outcomes in adult patients (>18 years) diagnosed with AL amyloidosis. Study Design: Retrospective observational study. Place and Duration of Study: Department of Hematology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India, between 2007-2018. Methodology: Retrospectively, 11 year follow-up data (2007-2018) was retrieved from the electronic medical records. Results: The study population had a median age of 52 years and 60.6% were males. Heart and kidneys were the most frequently affected organs. Cardiac involvement was the most important determinant of clinical outcome in patients with primary amyloidosis in general and was also associated with poor outcome in this cohort of patients. Of the 33 eligible patients included in the study, 9 patients underwent treatment. Bortezomib-based regimen (CyBorD) was the preferred first-line treatment (27.3% patients). Overall, 6.0% of the patients presented a deep response (complete or very good partial response). There is limited real-world evidence data from India regarding the prevalence and treatment outcomes for AL amyloidosis due to underdiagnosis and late presentation. Conclusion: This study provides vital real-world evidence of prevalence and treatment outcomes in primary amyloidosis patients in India.