Phase II Study Evaluating Maintenance in Light Chain Amyloidosis (EMILIA)
This phase II trial compares shorter-duration versus longer-duration maintenance therapy with daratumumab for improving survival in patients who have received initial treatment with daratumumab for light chain (AL) amyloidosis. Maintenance therapy is treatment that is given to help keep cancer from coming back after it has disappeared following initial therapy. Daratumumab is in a class of medications called monoclonal antibodies. It binds to a protein called CD38, which is found on some types of immune cells and cancer cells, including myeloma cells. Daratumumab may block CD38 and help the immune system kill cancer cells. Daratumumab is commonly prescribed as initial treatment for patients with AL amyloidosis. However, it is not known what role daratumumab may play in the maintenance therapy period of patients with AL amyloidosis. This phase II trial compares shorter duration maintenance to longer duration maintenance for improving survival in patients with AL amyloidosis.
• Age \>= 18 years
• Histological confirmation of AL amyloidosis with adequate typing (mass spectrometry, immunohistochemistry, immunofluorescence, immunogold)
• AL amyloidosis with organ disease requiring therapy
⁃ NOTE: Disease requiring therapy is referred to the time of diagnosis. There are no limitations in baseline measurable disease parameters
• Patients must have monoclonal protein studies (serum free light chain assay, serum immunofixation or serum MASS-FIX) obtained at time of diagnosis before induction therapy initiated and available for review to be enrolled.
⁃ NOTE: Patients are allowed to participate in this study if urine electrophoresis immunofixation study was not done at time of diagnosis or cannot be obtained
• Patients must have completed 6 cycles of daratumumab (Dara)-CyBorD-based induction treatment prior to registration or have a plan to complete cycle 6 prior to registration. NOTE: treatment in the study is allowed only after the completion of 6 cycles of induction
• Patients must have achieved a hematological complete response (CR) (irrespective of organ response achievement) or hematological very good partial response (VGPR) (irrespective of organ response achievement) or hematological low-difference in involved and uninvolved free light chain (dFLC) partial response (PR) (irrespective of organ response achievement) or hematological PR with at least one organ response after receiving Dara-CyBorD-based induction.
⁃ NOTE: Patients with baseline dFLC \< 5 mg/dL, must have achieved hematological CR, or dFLC \< 1 mg/dL or achieved organ response prior to randomization
• Patients in whom bortezomib and/or cyclophosphamide were omitted from induction due to toxicity concerns or adverse effects are allowed. Patients must receive at least daratumumab and dexamethasone at induction to qualify for the study
⁃ NOTE: Dexamethasone use does not need to be carried to end of induction for eligibility consideration
• Eastern Cooperative Oncology Group (ECOG) performance status (PS) 0, 1, 2 or 3
• Hemoglobin \>= 8.0 g/dL (obtained =\< 28 days prior to registration)
• Absolute neutrophil count (ANC) \>= 1000/mm\^3 (obtained =\< 28 days prior to registration)
• Platelet count \>= 50,000/mm\^3 (obtained =\< 28 days prior to registration)
• Negative pregnancy test done =\< 7 days prior to registration, for persons of childbearing potential only.
⁃ NOTE: If the urine test is positive or cannot be confirmed as negative, a serum pregnancy test will be required
• Provide written informed consent
⁃ NOTE: Informed consent required =\< 90 days prior registration
• Ability to complete questionnaire(s) by themselves or with assistance
• Willing to return to enrolling institution for follow-up (during the active monitoring phase of the study)