Daratumumab, lenalidomide and dexamethasone in newly diagnosed systemic light chain amyloidosis patients associated with multiple myeloma

Kawano, Yawara; Hata, Hiroyuki; Takashio, Seiji; Tsujita, Kenichi; Ueda, Mitsuharu; Matsuoka, Masao

doi:10.1111/bjh.18234

Cited by 4 publications

(2 citation statements)

References 15 publications

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“… 1 According to the phase 3 ANDROMEDA trial, which was recently published, treatment with daratumumab, cyclophosphamide, bortezomib, and dexamethasone is becoming the standard of care for patients with systemic primary amyloidosis. 9 Bortezomib is the preferred treatment in patients under renal replacement therapy as it does not require dose adjustment. 10…”

Section: Discussionmentioning

confidence: 99%

Primary Systemic Amyloidosis: A Case Report

Sen Oli,

Jha,

Karki

et al. 2023

J Nepal Med Assoc

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Primary systemic amyloidosis is a systemic disease characterised by the deposition of misfolded proteins extracellularly in different organs without any known cause in the background, eventually leading to multiorgan dysfunction and death. The incidence of primary amyloidosis is estimated at 5.1-12.8 cases per million, with a poor prognosis. We report a case of a 69-year male with lower back pain, shortness of breath, and anasarca diagnosed as primary systemic amyloidosis by serum-free light chain assay and kidney needle biopsy. He was started on intravenous bortezomib and dexamethasone. Though he adhered to his medications, with time he developed renal insufficiency marked by azotemia following which hemodialysis was performed. Primary systemic amyloidosis is a rare clinical condition with a very poor prognosis. Further studies are needed to understand the proper pathophysiology and treatment of the disease.

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Section: Discussionmentioning

confidence: 99%

Primary Systemic Amyloidosis: A Case Report

Sen Oli,

Jha,

Karki

et al. 2023

J Nepal Med Assoc

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“…Over 50% achieved a very good partial response. Eight patients had cardiac amyloid evaluable for cardiac response, and this was achieved in 4 93 …”

Section: Conventional Agents In the Treatment Of Amyloidosismentioning

confidence: 99%

Immunoglobulin light chain amyloidosis: 2024 update on diagnosis, prognosis, and treatment

Gertz

2023

American J Hematol

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Disease OverviewImmunoglobulin light chain amyloidosis is a clonal, nonproliferative plasma cell disorder in which fragments of immunoglobulin light or heavy chain are deposited in tissues. Clinical features depend on organs involved but can include heart failure with preserved ejection fraction, nephrotic syndrome, hepatic dysfunction, peripheral/autonomic neuropathy, and “atypical smoldering multiple myeloma or MGUS.”DiagnosisTissue biopsy stained with Congo red demonstrating amyloid deposits with apple‐green birefringence is required for the diagnosis of AL amyloidosis. Organ biopsy is not required in 85% of patients. Verification that amyloid is composed of immunoglobulin light chains is mandatory. The gold standard is laser capture mass spectroscopy.PrognosisN‐terminal pro–brain natriuretic peptide (NT‐proBNP or BNP), serum troponin T(or I), and difference between involved and uninvolved immunoglobulin free light chain values are used to classify patients into four stages; 5‐year survivals are 82%, 62%, 34%, and 20%, respectively.TherapyAll patients with a systemic amyloid syndrome require therapy to prevent deposition of amyloid in other organs and prevent progressive organ failure. Current first‐line therapy with the best outcome is daratumumab, bortezomib, cyclophosphamide, and dexamethasone. The goal of therapy is a ≥VGPR. In patients failing to achieve this depth of response options for consolidation include pomalidomide, stem cell transplantation, venetoclax, and bendamustine.Future ChallengesDelayed diagnosis remains a major obstacle to initiating effective therapy prior to the development of end‐stage organ failure. Trials of antibodies to deplete deposited fibrils are underway.

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