Amyloidosis

Questions discussed in this category

How do you approach patients with AL amyloidosis with t(11;14) who have not achieved a hematologic complete response to induction therapy with 6 cycles of Dara-CyBorD?

Specifically, in the setting of patients that have had a PR or VGPR and are not actively progressing.

2 Answers available

How do you approach initial treatment for AL amyloidosis with excess plasmacytosis in the bone marrow, that has cardiac involvement and is not a transplant candidate?

Let's assume they have cardiac involvement and transplant may not be feasible.

1 Answer available

How do you approach management of a patient with Sjogren's disease and lung mass biopsy revealing amyloidosis?

1 Answer available

How extensive of a workup do you do for systemic AL amyloidosis for a patient with a resected amyloidoma, localized AL amyloid deposition in their airway or lungs, who has no other symptoms?

Would you do a 24-hour urine and echocardiogram in all of these patients? Cardiac biomarkers, PT/PTT, or any other such blood tests?

3 Answers available

Would testing for ATTR cardiac amyloidosis be considered in an older patient with bilateral carpal tunnel surgeries and multiple spinal stenosis surgeries, but no obvious cardiac symptoms?

1 Answer available

What would be your index of suspicion for AL amyloidosis with cardiac involvement in a patient with MGUS to prompt further imaging (and which modality would be preferred)?

1 Answer available

What is your approach to maintenance therapy post autologous stem cell transplant for patients with concurrent multiple myeloma and AL amyloidosis?

Does your choice of agent differ, or are you more or less likely to offer maintenance than if the patient had multiple myeloma alone?

2 Answers available

Would you substitute carfilzomib in place of bortezomib in a transplant-eligible AL amyloidosis patient with baseline amyloid neuropathy and worsening neuropathy on bortezomib despite maximal dose reduction?

2 Answers available

What induction regimen would you choose for a patient with newly diagnosed multiple myeloma with incidental amyloidosis noted on bone marrow biopsy, but with no end-organ damage as a result of amyloid deposition?

Would you consider Dara-CyBorD or a MM triplet/quadruplet such as VRd or Dara-VRd?

4 Answers available

How do you approach treating a patient with IgM-AL amyloidosis?

Given the rarity of this diagnosis (5-7% of all AL amyloidosis cases), and the prognostic and clinical differences when compared to non IgM-AL am...

3 Answers available

What options are available for patients with relapsed/refractory AL amyloidosis after Dara-CyBorD, other than clinical trial?

1 Answer available

What is your approach to a patient with systemic AL amyloidosis who has achieved a hematologic response but not an organ response with CyBorD?

Would you proceed to autologous transplant, or switch to an alternate regimen (eg. daratumumab-based) first? Would the type or degree of organ involv...

1 Answer available

How do you approach the workup and initial treatment of systemic AL amyloidosis?

Once a tissue biopsy has confirmed light chain amyloid, what additional tests do you perform as part of a standard workup? In whom should treatment b...

1 Answer available

Papers discussed in this category

N Engl J Med,

Daratumumab-Based Treatment for Immunoglobulin Light-Chain Amyloidosis.

Journal of clinical oncology : official journal of the American Society of Clinical Oncology, 1993-05

Primary systemic amyloidosis: a rare complication of immunoglobulin M monoclonal gammopathies and Waldenström's macroglobulinemia.

British journal of haematology, 2018-10

Targeting CD38 with daratumumab is lethal to Waldenström macroglobulinaemia cells.

Blood Adv,

Multicenter phase 2 study of daratumumab monotherapy in patients with previously treated Waldenström macroglobulinemia.

Blood Cancer J, 2020 May 11

Venetoclax for the treatment of translocation (11;14) AL amyloidosis.

Lancet Oncol, 2020 Oct 29

Venetoclax or placebo in combination with bortezomib and dexamethasone in patients with relapsed or refractory multiple myeloma (BELLINI): a randomised, double-blind, multicentre, phase 3 trial.

Blood Cancer J, 2021 Jan 11

Venetoclax induces deep hematologic remissions in t(11;14) relapsed/refractory AL amyloidosis.

Blood, 2016 Mar 21

VTD is superior to VCD prior to intensive therapy in multiple myeloma: results of the prospective IFM2013-04 trial.

Amyloid, 2017 Apr 23

Amyloid in bone marrow smears in systemic light-chain amyloidosis.

Leukemia, 2015 Oct 07

Bortezomib-thalidomide-dexamethasone (VTD) is superior to bortezomib-cyclophosphamide-dexamethasone (VCD) as induction therapy prior to autologous stem cell transplantation in multiple myeloma.

British journal of haematology, 2017-10

Natural history of amyloidosis isolated to fat and bone marrow aspirate.

Blood Adv,

Primary treatment of light-chain amyloidosis with bortezomib, lenalidomide, and dexamethasone.

Blood Cancer J, 2018 May 24

Evaluation of minimal residual disease using next-generation flow cytometry in patients with AL amyloidosis.

Amyloid, 2019 Jan 20

Efficacy of lenalidomide as salvage therapy for patients with AL amyloidosis.

Blood advances, 2018-05-22

Epidemiology of AL amyloidosis: a real-world study using US claims data.

JACC CardioOncol, 2020 Mar 17

Plasma Hepatocyte Growth Factor for Diagnosis and Prognosis in Light Chain and Transthyretin Cardiac Amyloidosis.

Mayo Clin Proc, 2010 Aug 16

Prevalence of monoclonal gammopathy of undetermined significance: a systematic review.

ESC Heart Fail, 2021 Dec 08

Echocardiographic and clinical predictors of cardiac amyloidosis: limitations of apical sparing.

American journal of hematology, 2022 Jul 05

Predictors of hematologic response and survival with stem cell transplantation in AL amyloidosis: A 25-year longitudinal study.

Blood advances, 2023 Oct 24

Predictors of treatment response and survival outcomes in patients with advanced cardiac AL amyloidosis.

Blood, 2018 Aug 14

Treatment of AL amyloidosis with bendamustine: a study of 122 patients.

Leukemia, 2024 Apr 09

Treatment patterns for AL amyloidosis after frontline daratumumab, bortezomib, cyclophosphamide, and dexamethasone treatment failures.

Haematologica, 2013-01

Amyloidosis of the gastrointestinal tract: a 13-year, single-center, referral experience.

The Lancet. Haematology, 2015 May 06

Natural history and outcomes in localised immunoglobulin light-chain amyloidosis: a long-term observational study.

European respiratory review : an official journal of the European Respiratory Society, 2017 Sep 06

The lung in amyloidosis.

Mayo Clinic proceedings, 2017-06

Presentation and Outcomes of Localized Immunoglobulin Light Chain Amyloidosis: The Mayo Clinic Experience.

Blood Cancer J, 2022 Sep 14

Defining new reference intervals for serum free light chains in individuals with chronic kidney disease: Results of the iStopMM study.

Arthritis Rheumatol, 2021 Jul

2021 American College of Rheumatology Guideline for the Treatment of Rheumatoid Arthritis.

Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis, 2021 Nov 12

Tissue biopsy for the diagnosis of amyloidosis: experience from some centres.

Radiographics : a review publication of the Radiological Society of North America, Inc, 2013 Nov-Dec

CT findings of thoracic manifestations of primary Sjögren syndrome: radiologic-pathologic correlation.

Amyloidosis

Questions discussed in this category

Papers discussed in this category

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