Clinical Characteristics of Light Chain Amyloidosis in an Underserved Patient Population

Background

Primary or light chain (AL) amyloidosis is the most common form of systemic amyloidosis in which insoluble fibrils are deposited in various tissues and can ultimately lead to multi-organ failure. It is commonly linked to plasma cell dyscrasias which lead to the overproduction and deposition of immunoglobin proteins. Due to multi-organ involvement and difficulty in diagnosing patients with light chain amyloidosis, these patients are faced with greater challenges present with extensive disease especially in an underserved patient population in the Bronx. Our aim was to report clinical characteristics of patients at a tertiary care center in the Bronx

Methods

A retrospective analysis of 29 patients with AL amyloidosis were evaluated at Montefiore Medical Center from 2008 until 2023 was performed to determine demographics, clinical characteristics, and outcome measures. Patient demographics such as age, sex, and race/ethnicity as well as organ involvement, mode of diagnosis, and disease staging were determined. The Mayo 2004 staging system was used for staging at the time of diagnosis.

Results

29 patients with AL amyloidosis were identified at Montefiore Medical center from 2008-2023, the median age of diagnosis was 63 (range 36-87). There were 18 men (62%) and 11 women (38%). Most of the female and male patients were black with 5 (45%) and 9 (50%) respectively. One third of the population were Hispanic. Out of all the patients, 11 (38%) had positive bone marrow biopsies at time of diagnosis and the most common organ involvement was cardiac with 21 patients at 72% of the data set. 11 had renal involvement (38%) and 9 (31%) had involvement of the gastrointestinal tract. 11 of the patients were stage 3 disease (38%) and 9 patients had stage 1 and 2 disease (31%) each. Majority of patients had lambda light chain involvement (55%). 2 patients had concurrent TTR amyloidosis. In terms of follow-up, 4 patients have died while 4 patients have been lost to follow-up.

Conclusion

Our study reports the extensive organ involvement in our patient population. A third of the patients present with Stage III AL amyloidosis with most patients having cardiac involvement. We aim to report specific outcomes for this patient population at the meeting.