AL amyloidosis associated with B-cell lymphoproliferative disorders: frequency and treatment outcomes

AL amyloidosis, a systemic disorder characterized by widespread deposition of amyloid fibrils derived from monoclonal Ig light chains in organs and soft tissues, is typically caused by an underlying plasma cell dyscrasia. However, this disease can also be associated rarely with a B-cell lymphoproliferative disorder. In this report, we describe the presentation and clinical course of 16 patients with this association. Although amyloid-related organ involvement in these patients was typical of AL amyloidosis, the patients in this series were on average older and more likely to be female than patients with disease associated with a plasma cell dyscrasia. They were also more likely to have multisystem involvement. Treatment decisions were based primarily on the dominent hematopathologic features of the associated lymphoproliferative disorder. However, high-dose melphalan and stem cell transplantation was the primary therapy in 5 patients, and each of these patients had prolonged survival, ranging from 36 to 102 months.