Primary amyloidosis medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shyam Patel [2]

Overview

Patients with systemic AL amyloidosis are not cured with conventional immunosuppressant treatment. However, early mortality rates have decreased and survival has improved as there has been a shift toward earlier diagnosis and therapy aimed at achieving remissions. The most commonly used regimen for AL amyloidosis is CyBorD, which consists of cyclophosphamide, bortezomib, and dexamethasone.

Medical Therapy

Patients with systemic AL amyloidosis are not cured with immunosuppressant conventional treatment. However, early mortality rates have decreased and survival has improved as there has been a shift toward earlier diagnosis and therapy aimed at achieving deep remissions

Patients with newly diagnosed primary amyloidosis should be assessed to determine their eligibility for autologous hematopoietic cell transplantation.

Patients must fulfill all eligibility criteria to be able to undergo transplantation:[1]

Some patients with primary amyloidosis respond to chemotherapy focused on the abnormal plasma cells. A stem cell transplant may be done, as in multiple myeloma.

The most commonly used regimen for AL amyloidosis is CyBorD, which consists of cyclophosphamide, bortezomib, and dexamethasone.[2]

Therapy Mechanism of Action Dosing Adverse Effects

Bortezomib

  • Cycles 1-4: 1.3mg/m2 IV/SC on days 1, 4, 8, 11, 22, 25, 29, 32
  • Cycles 5-9: 1.3mg/m2 IV/SC on days 1, 8, 22, 29

Peripheral neuropathy, VZV reactivation, hepatic impairment, asthenia, diarrhea, nausea, constipation, arthralgia, edema, dizziness

Dexamethasone

  • 40mg PO weekly

Infections, immunosuppression, bone loss, cataract formation, glaucoma, muscular atrophy

Melphalan

  • 6mg PO daily for 2-3 weeks, OR
  • 10mg PO daily for 7-10 days, OR
  • 0.15mg/kg daily PO for 7 days, THEN
  • 1-3mg or 0.05mg/kg PO daily after counts recover

Myelosuppression, nausea, vomiting, pulmonary fibrosis, stomatitis

Cyclophosphamide

  • 40-50mg/kg weekly

Myelosuppression, nausea, vomiting, hemorrhagic cystitis, secondary malignancies

Patisiran[3]

  • 0.3mg/kg weekly

Dyspepsia, dyspnea, erythema, bronchitis, blurry vision

Daratumumab[4]

  • Anti-CD38 monoclonal antibody
  • Depletes B lymphocytes and plasma cells
  • 16mg/kg weekly for weeks 1-8, then every 2 weeks for weeks 9-24, then every 4 weeks thereafter

Anemia, neutropenia, false positive indirect Coomb's test, infusion reaction, lymphopenia

References

  1. Gertz MA (2012). "How to manage primary amyloidosis". Leukemia. 26 (2): 191–8. doi:10.1038/leu.2011.219. PMID 21869840.
  2. Milani P, Merlini G, Palladini G (2018). "Novel Therapies in Light Chain Amyloidosis". Kidney Int Rep. 3 (3): 530–541. doi:10.1016/j.ekir.2017.11.017. PMC 5976806. PMID 29854961.
  3. Adams D, Suhr OB, Dyck PJ, Litchy WJ, Leahy RG, Chen J; et al. (2017). "Trial design and rationale for APOLLO, a Phase 3, placebo-controlled study of patisiran in patients with hereditary ATTR amyloidosis with polyneuropathy". BMC Neurol. 17 (1): 181. doi:10.1186/s12883-017-0948-5. PMC 5594468. PMID 28893208.
  4. van de Donk NW, Janmaat ML, Mutis T, Lammerts van Bueren JJ, Ahmadi T, Sasser AK; et al. (2016). "Monoclonal antibodies targeting CD38 in hematological malignancies and beyond". Immunol Rev. 270 (1): 95–112. doi:10.1111/imr.12389. PMC 4755228. PMID 26864107.

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