Localized Amyloidosis of Palate

Chandra, Ananya; Khan, Sumana; Dhabal, Arunima; Banerjee, Gautam

doi:10.4103/idoj.idoj_405_21

Cited by 2 publications

(1 citation statement)

References 9 publications

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“…However, there are a few exceptions, like localized AL Amyloidosis. Localized AL Amyloidosis is generally present in a single organ, such as the lungs, part of the bowel, upper airways, or skin along with the absence of the monoclonal protein in the plasma and absence of clonal plasma cells in the bone marrow [ 15 – 17 ]. Localized AL amyloidosis is not treated by systemic therapy since the long-term prognosis is favorable, and not complicated with systemic AL amyloidosis and is generally managed by local resection [ 18 ].…”

Section: Indications For Systemic Therapymentioning

confidence: 99%

Systemic AL amyloidosis: current approach and future direction

et al. 2023

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Systemic Light chain (AL) amyloidosis is a monoclonal plasma cell proliferative disorder characterized by deposition of amyloidogenic monoclonal light chain fragments causing organ dysfunction. It is a fatal disease and if not diagnosed and treated early can lead to organ failure and potentially death. The renal system along with the cardiovascular system are the most common organs involved but other organs such as gut and liver can be involved as well. The initial evaluation of patients requires confirming the diagnosis with tissue biopsy and staining with Congo red followed by confirmatory typing with mass spectrometry of the Congo red positive tissue. Then establishing the extent of the organs involvement by various staging and biomarkers testing. The treatment options and the tolerability of therapy depend on the disease staging, frailty, and co-morbidities. The autologous hematopoietic cell transplantation (HCT) after high dose melphalan therapy is an effective strategy which is usually done after initial bortezomib induction therapy. Unfortunately, most systemic AL amyloidosis patients are not candidate for HCT due to frailty, old age, multi-organ involvement, renal and heart failure at the time of diagnosis. While it is widely accepted that the patients need to be treated until they achieve complete hematologic response, the maintenance therapy after HCT is not well established in AL amyloidosis. In this review, we report the literature on the latest treatment updates of AL amyloidosis and the ongoing clinical trials highlighting the future treatments.

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Section: Indications For Systemic Therapymentioning

confidence: 99%