Immunoglobulin-Related Amyloidosis Medication

Updated: Nov 30, 2019
  • Author: Slavomir Urbancek, MD, PhD; Chief Editor: Emmanuel C Besa, MD  more...
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Medication

Medication Summary

Melphalan plus prednisone is considered standard therapy for L chain–type amyloidosis, with any myeloma regimen offering a reasonable therapeutic choice.

Diuretics are the mainstay of therapy for L chain–type amyloidosis–related congestive heart failure. The optimal degree of diuresis is often difficult to judge. When edema is troubling and symptomatic postural hypotension is not present, fluid can be removed with careful diuresis. Conversely, hypotension resulting from a low ejection fraction and/or autonomic neuropathy may limit diuretic use.

Digoxin and calcium channel blockers are contraindicated in cardiac amyloidosis, because these agents bind to amyloid fibrils, which may worsen heart failure and produce arrhythmias.

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Immunosuppressive Agents

Class Summary

Two slightly different regimens of melphalan and prednisone have been used in 2 large studies. Either regimen can be used to treat this condition.

Melphalan (Alkeran) and prednisone (Deltasone, Orasone, Meticorten)

Melphalan reduces clonal plasma cell population. Inhibits mitosis by cross-linking DNA strands. Individual tolerance to melphalan varies. Adjust dosage after the first cycle, based on the degree of cytopenia in the previous cycles. Nadir counts appear 2-3 wk following administration. Should be taken on an empty stomach. Prednisone reduces clonal plasma cell population.

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Antineoplastic Agent, Proteasome Inhibitor

Class Summary

Proteasome inhibitors are antineoplastic agents that inhibit cell growth and proliferation.

Bortezomib (Velcade)

First drug approved of anticancer agents known as proteasome inhibitors. The proteasome pathway is an enzyme complex existing in all cells. This complex degrades ubiquitinated proteins that control the cell cycle and cellular processes and maintains cellular homeostasis. Reversible proteasome inhibition disrupts pathways supporting cell growth, thus decreases cancer cell survival.

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Immunosuppressant Agents

Class Summary

Immunosuppressant agents may suppress the production of factors that mediate immune reactions.

Thalidomide (Thalomid)

Immunomodulatory agent that may suppress excessive production of tumor necrosis factor-alpha (ie, TNF-alpha) and may downregulate selected cell-surface adhesion molecules involved in leukocyte migration. Because of concerns regarding teratogenicity, thalidomide can only be prescribed by registered physicians and dispensed by registered pharmacists. Patients must participate in ongoing surveys to receive therapy, and only a 28-day supply can be prescribed at a time. Indicated in conjunction with dexamethasone to treat newly diagnosed multiple myeloma.

Lenalidomide (Revlimid)

Indicated for transfusion-dependent MDS subtype of deletion 5q cytogenetic abnormality. Structurally similar to thalidomide. Elicits immunomodulatory and antiangiogenic properties. Inhibits proinflammatory cytokine secretion and increases anti-inflammatory cytokines from peripheral blood mononuclear cells.

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