Mini Tutorial – AL Amyloidosis

Instructions:- Hover the curser over PPT below so that BUTTON panel appears in lower portion of PPT and use to navigate as shown in image above !!

High yield points are also given below!!

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NOTE > If using iPhone or iPad kindly use browser other than safari as  the box would not load in safari.

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

1. Physiologic age 70 years or younger
2. Performance score of 2 or less
3. Troponin T level less than 0.06 ng/Ml
4. Systolic blood pressure of at least 90 mm Hg
5. Creatinine clearance of at least 30 mL/min (unless undergoing long-term dialysis)
6. New York Heart Association class I/II
7. 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)