Retrospective Analysis of AL Amyloidosis

A consecutive series of 142 newly diagnosed AL amyloidosis patients, who presented to our hospital from November 2012 to December 2021, were analyzed retrospectively. A total of 80 of the 142 newly diagnosed AL amyloidosis patients had primary AL amyloidosis, and the other 62 patients were AL amyloidosis patients with concurrent MM. The diagnosis of AL amyloidosis was confirmed by a Congo-red-positive biopsy and immunohistochemistry of the amyloid [1 (link)]. Meanwhile, the presence of the kappa or lambda chain was confirmed through immunohistochemistry and/or immunofluorescence. Moreover, monoclonal plasma cell proliferation was investigated by multi-color flow cytometry on aspirated bone marrow cells. Patients were evaluated for the presence of symptomatic MM based on the International Myeloma Working Group (IMWG) criteria [11 (link)]: bone marrow clonal plasma cells > 10% and evidence of myeloma-defining events, including the slim-CRAB criteria. Patients with AL amyloidosis with >10% bone marrow plasma cells and any myeloma-defining events were described with coexistent MM. iFISH was performed for all the patients as part of their routine clinical testing. Patients gave written informed consent for the iFISH and data analysis in accordance with the Declaration of Helsinki. Approval was obtained by the Ethics Committee of Shanghai Changzheng Hospital.
Among the 80 primary AL amyloidosis patients, 71 patients were treated with bortezomib-based regimens (cyclophosphamide/bortezomib/dexmethasome (CBd) or bortezomib/dexamethasone (Vd)), five patients treated with lenalidomide-based regimens (lenalidomide/cyclophosphamide/dexmethasome (RCd) or lenalidomide/dexmethasome (Rd)), three patients were treated with thalidomide-based regimens (thalidomide/ cyclophosphamide/dexmethasome (TCd)), and one patient was treated with ixazomib-based regimens (ixazomib/dexmethasome (Id)) for induction therapy, and three of the 80 patients received autologous transplants as a consolidation therapy. Among the 62 AL amyloidosis patients with concurrent MM, 47 patients were treated with bortezomib-based regimens (CBd or Vd), seven patients were treated with lenalidomide-based regimens (RCd or Rd), five patients were treated with bortezomib combined with lenalidomide (bortezomib/lenalidomide/dexmethasome (VRd)), and three patients were treated with daratumumab-based regimens (daratumumab/bortezomib/dexmethasome (DVd)). A total of six of the 62 patients received autologous transplants as a consolidation therapy. None of the 142 patients received venetoclax.