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High yield points are also given below!!
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Treatment Goals of AL-Amyloidosis
- Guideline. The goal of treatment should be a hematologic response of very good partial response (VGPR) or better.
- Guideline. The goal of treatment (if cardiac involvement) should be a 30% reduction in NT-proBNP or better.
Autologous Stem Cell Transplant
- Guideline. Consider high-dose chemotherapy with ASCT in selected patients.
- Guideline. Select candidates for ASCT based on troponin level, blood pressure, renal function, and physiologic age.
To be transplant eligible, the following criteria should be met
- Physiologic age 70 years or younger
- Performance score of 2 or less
- Troponin T level less than 0.06 ng/Ml
- Systolic blood pressure of at least 90 mm Hg
- Creatinine clearance of at least 30 mL/min (unless undergoing long-term dialysis)
- New York Heart Association class I/II
- No more than 2 major organs significantly involved (liver, heart, kidney, or autonomic nerve).
- Guideline. Dose-attenuated conditioning chemotherapy with ASCT for sicker patients is not recommended outside of a clinical trial.
- Guideline. Induction therapy before ASCT for patients with less than 10% bone marrow plasmacytosis (AL amyloidosis without MM) is not universally recommended.
- Guideline. Induction therapy before ASCT for patients with 10% or greater bone marrow plasmacytosis (AL amyloidosis with MM) is recommended.
- Guideline. Consolidation therapy for patients eligible for SCT can be considered in patients not achieving a VGPR or better.
- Guideline. Maintenance therapy after ASCT is not recommended outside of clinical trials.
- Guideline. Allogeneic hematopoietic SCT is not recommended outside of clinical trials.
- Guideline for ASCT for Patients Undergoing Hemodialysis. An ASCT is feasible, especially if renal allograft is being considered.
BJH Guideline for chemotherapy: –
- First line treatment is recommended with combination chemotherapy regimens similar to those used in myeloma but typically using dexamethasone.
- Proteasome inhibitor-based regimens are a preferred choice due to better response rates and outcomes in phase II studies and a bortezomib-alkylator-steroid (CyBorD) combination is preferred where a rapid response desirable (cardiac involvement, renal impairment, severe hypoalbuminaemia, fluid retention)