Primary amyloidosis medical therapy: Difference between revisions
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Patients must fulfill all eligibility criteria to be able to undergo transplantation:<ref name="pmid21869840">{{cite journal| author=Gertz MA| title=How to manage primary amyloidosis. | journal=Leukemia | year= 2012 | volume= 26 | issue= 2 | pages= 191-8 | pmid=21869840 | doi=10.1038/leu.2011.219 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21869840 }}</ref> | Patients must fulfill all eligibility criteria to be able to undergo transplantation:<ref name="pmid21869840">{{cite journal| author=Gertz MA| title=How to manage primary amyloidosis. | journal=Leukemia | year= 2012 | volume= 26 | issue= 2 | pages= 191-8 | pmid=21869840 | doi=10.1038/leu.2011.219 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21869840 }}</ref> | ||
* Age ≤70 years | *[[Age]] ≤70 years | ||
** There is no strict age limit, decisions are made on a case-by-case basis. | ** There is no strict age limit, decisions are made on a case-by-case basis. | ||
* Troponin T < 0.06 ng/mL | *[[Troponin]] T < 0.06 ng/mL | ||
* Systolic blood pressure ≥ 90 mmHg | *[[Systolic blood pressure]] ≥ 90 mmHg | ||
* Creatinine clearance ≥ 30 mL/min | *[[Creatinine clearance]] ≥ 30 mL/min | ||
** Unless patient is on chronic stable dialysis | ** Unless patient is on chronic stable dialysis | ||
* Eastern Cooperative Oncology Group (ECOG) performance status ≤ 2 | *[[Eastern Cooperative Oncology Group|Eastern Cooperative Oncology Group (ECOG)]] performance status ≤ 2 | ||
* NYHA functional status class I or II | *[[New york heart association functional classification|NYHA functional status class]] I or II | ||
* No more than 2 involved organs | * No more than 2 involved [[organs]] | ||
* No large pleural effusions | * No large [[pleural effusions]] | ||
* No dependency on oxygen therapy | * No dependency on [[oxygen therapy]] | ||
Some patients with primary [[amyloidosis]] respond to [[chemotherapy]] focused on the abnormal [[plasma cell]]s. A [[stem cell transplant]] may be done, as in [[multiple myeloma]]. | Some patients with primary [[amyloidosis]] respond to [[chemotherapy]] focused on the abnormal [[plasma cell]]s. A [[stem cell transplant]] may be done, as in [[multiple myeloma]]. | ||
Latest revision as of 15:55, 31 October 2019
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Primary amyloidosis Microchapters |
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Treatment |
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Case Studies |
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Primary amyloidosis medical therapy On the Web |
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American Roentgen Ray Society Images of Primary amyloidosis medical therapy |
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Risk calculators and risk factors for Primary amyloidosis medical therapy |
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]
- Age ≤70 years
- There is no strict age limit, decisions are made on a case-by-case basis.
- Troponin T < 0.06 ng/mL
- Systolic blood pressure ≥ 90 mmHg
- Creatinine clearance ≥ 30 mL/min
- Unless patient is on chronic stable dialysis
- Eastern Cooperative Oncology Group (ECOG) performance status ≤ 2
- NYHA functional status class I or II
- No more than 2 involved organs
- No large pleural effusions
- No dependency on oxygen therapy
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 initial step in the treatment of this disorder is to correct the organ failure, since the disease is discovered at an advanced stage when multiple organ systems may be affected.
- Nephrotic syndrome is treated using supportive therapy and diuretics.
- Renal failure is treated with dialysis.
- Heart failure is treated using diuretics.
- Gastrointestinal and nerve involvement are treated symptomatically.
The most commonly used regimen for AL amyloidosis is CyBorD, which consists of cyclophosphamide, bortezomib, and dexamethasone.[2]
References
- ↑ Gertz MA (2012). "How to manage primary amyloidosis". Leukemia. 26 (2): 191–8. doi:10.1038/leu.2011.219. PMID 21869840.
- ↑ 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.
- ↑ 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.
- ↑ 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.