1,721,211 research outputs found
When should treatment of AL amyloidosis start at relapse? Early, to prevent organ progression
Two-hit strategy for treating AL amyloidosis?
In this issue of Blood, Edwards et al report the results of a phase 1a/1b trial of passive antiamyloid immunotherapy with the monoclonal antibody CAEL-101 in patients with light chain (LC) amyloidosis with persistent organ dysfunction despite response to previous chemotherapy. Two-thirds of evaluable patients attained cardiac response and 20% reached renal response at a median time of only 3 weeks. Treatment was well tolerated. These findings open new therapeutic opportunities and create new challenges.(1
The quest for validated treatment endpoints in light chain (AL) amyloidosis: composite criteria for a composite disease
[Histochemical studies on the mucocartilage of the lamprey during its larval ontogenesis].
Conventional Therapy for Amyloid Light-Chain Amyloidosis
The vast majority of patients with light-chain (AL) amyloidosis are not eligible for stem cell transplant and are treated with conventional chemotherapy. Conventional regimens are based on various combinations of dexamethasone, alkylating agents, proteasome inhibitors, and immunomodulatory drugs. The choice of these regimens requires a careful risk stratification, based on the extent of amyloid organ involvement, comorbidities, and the characteristics of the amyloidogenic plasma cell clone. Most patients are treated upfront with bortezomib and dexamethasone combined with cyclophosphamide or melphalan. Cyclophosphamide does not compromise stem cell mobilization and harvest and is more manageable in renal failure. Melphalan can overcome the effect of t(11;14), which is associated with lower response rates and shorter survival in subjects treated with bortezomib and dexamethasone, or in combination with cyclophosphamide. Lenalidomide and pomalidomide are the mainstay of rescue treatment. They are effective in patients exposed to bortezomib, dexamethasone, and alkylators, but deep hematologic responses are rare. Ixazomib, alone or in combination with lenalidomide, increases the rate of complete responses in relapsed/refractory patients. Conventional chemotherapy regimens will represent the backbone for future combinations, particularly with anti-plasma-cell immunotherapy, that will further improve response rates and outcomes
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