AN EXPERIENCE OF PRIMARY AMYLOIDOSIS FROM AN INDIAN TERTIARY CANCER INSTITUTE IN SOUTH INDIA

Abstract

Introduction: Primary or AL (amyloid Light chain) Amyloidosis is a clonal plasma cell disorder. It is an uncommon disorder and exact incidence is unknown. AL amyloidosis can present with variety of symptoms and signs. There are limited studies about clinical prole, treatment and outcomes of patients with AL Amyloidosis in Indian context. We aim to describe the clinical prole and outcomes of patients with AL amyloidosis in our center. A retrospective descriptive analysis was carried out on patients Materials & Methods: with proven AL amyloidosis, treated from October 2013 to August 2022. Data from 11 patients was compiled and analyzed. The median Results: age at presentation was 54 years. All patients were males (100%). Most common symptoms were pedal oedema in 8 (72%) patients, shortness of breath in 4(36%) patients, Loss of sensations and tingling of upper and lower limbs in 2(18%) patients and lymphadenopathy in 1(9%) patient. Kidney was involved in 7(63%) patients, heart in 3(27%) patients, peripheral nerve in 2(18%) patients and mediastinum in 1(9%) patient. The induction chemotherapy regimens used were Bortezomib-based in 8(72%) patients, Immunomodulator-based in 2(18%) patients. 2(18%) patients underwent Autologous Stem Cell Transplantation (ASCT). Patients who received bortezomib-based treatment had favourable haematological response (62.5%) compared to those who received non-bortezomib-based treatment (9%). Of 11 patients, 3(28%) patients died and 8(72%) patients are alive. This study Conclusion: presents the spectrum of clinical manifestations and outcomes in Indian population. Our study results are in common with the fact that response rates and survival improve with early diagnosis and treatment. Autologous stem cell transplantation should be considered for transplant eligible patients.