Abstract
Introduction
IgD-associated AL amyloidosis is a rare subtype, accounting for 1–2% of cases, with fewer than 100 reported. Prior studies show inconsistent findings on organ involvement, progression, and survival, underscoring the need for real-world data.
Methods
We performed a retrospective review of patients with IgD-associated AL amyloidosis at a tertiary referral center.
Results
Ten patients (0.6% of systemic AL cases) were identified. All evaluable patients had lambda light chain predominance; nine had detectable IgD lambda monoclonal protein. Cardiac involvement occurred in six patients and renal involvement in four; two had both. Most received upfront combination therapy, including autologous stem cell transplantation. Hematologic responses were favorable, though organ responses were less frequent. Median overall survival was 9.7 years.