Systemic Amyloidosis due to Low-Grade Lymphoma

Wechalekar, Ashutosh; Chakraborty, Rajshekhar; Lentzsch, Suzanne

doi:10.1016/j.hoc.2020.08.016

Cited by 9 publications

(10 citation statements)

References 45 publications

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“…Patients with AL amyloidosis diagnosed between January 1980 and December 2019 were identified from the prospectively maintained database at the Amyloidosis Center at Boston University School of Medicine and Boston Medical Center. Those with localized AL amyloidosis, myeloma-associated AL amyloidosis, and B cell lymphoproliferative disorder-associated AL amyloidosis were excluded from analysis due to differing risks and treatment approaches [ 30 – 35 ]. When necessary, typing of amyloidogenic protein was performed by immunohistochemistry, immunogold electron microscopy (IG-EM) or liquid chromatography and tandem mass spectrometry (LCMS 2 ).…”

Section: Methodsmentioning

confidence: 99%

Marked progress in AL amyloidosis survival: a 40-year longitudinal natural history study

et al. 2021

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The recent decades have ushered in considerable advancements in the diagnosis and treatment of systemic light chain (AL) amyloidosis. As disease outcomes improve, AL amyloidosis-unrelated factors may impact mortality. In this study, we evaluated survival trends and primary causes of death among 2337 individuals with AL amyloidosis referred to the Boston University Amyloidosis Center. Outcomes were analyzed according to date of diagnosis: 1980-1989 (era 1), 1990-1999 (era 2), 2000-2009 (era 3), and 2010-2019 (era 4). Overall survival increased steadily with median values of 1.4, 2.6, 3.3, and 4.6 years for eras 1–4, respectively (P < 0.001). Six-month mortality decreased over time from 23% to 13%. Wide gaps in survival persisted amid patient subgroups; those with age at diagnosis ≥70 years had marginal improvements over time. Most deaths were attributable to disease-related factors, with cardiac failure (32%) and sudden unexpected death (23%) being the leading causes. AL amyloidosis-unrelated mortality increased across eras (from 3% to 16% of deaths) and with longer-term survival (29% of deaths occurring >10 years after diagnosis). Under changing standards of care, survival improved and early mortality declined over the last 40 years. These findings support a more optimistic outlook for patients with AL amyloidosis.

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Section: Methodsmentioning

confidence: 99%

Marked progress in AL amyloidosis survival: a 40-year longitudinal natural history study

et al. 2021

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“…The optimal treatment for lymphoproliferative neoplasms associated with amyloidosis is chemotherapy or immunotherapy directed at the clonal cells. 6 However, such treatment generally aims to prevent further amyloid fibril deposition, rather than lead to substantial regression of deposits. In this patient’s case, she had end-stage heart failure related to constrictive pericarditis at the time of diagnosis, without any good therapeutic options.…”

Section: Discussionmentioning

confidence: 99%

Constrictive Pericarditis Revealing Rare Case of ALH Amyloidosis With Underlying Lymphoplasmacytic Lymphoma (Waldenstrom Macroglobulinemia)

O’Sullivan

Collins

et al. 2022

JACC: Case Reports

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“…When low-grade NHL have localized involvement, they tend to have low to undetectable circulating serum and urine levels of monoclonal protein, thus making this entity a diagnostic challenge. 10 Accurate identification of the underlying low-grade NHL and its type is important in initiating management.…”

Section: Discussionmentioning

confidence: 99%

The 13-year bleed: Exuberant amyloid angiopathies, angiodysplasias, and acquired coagulopathies of the gut

Simms

Suarez

Deeb

et al. 2021

SAGE Open Medical Case Reports

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Amyloidosis is a disorder characterized by extracellular deposits of proteins that are prone to aggregate and form insoluble fibrils. Amyloid deposits limited to a single organ or tissue without the involvement of any other site in the body is uncommon. We report a 75-year-old man with previously treated non-Hodgkin’s lymphoma who presented with recurrent gastrointestinal hemorrhage. Histopathology showed amyloid deposition within vascular malformations. His bleeding continued with the cause rooted in the fundamental building blocks—clotting factors. We discuss the interplay of the pathophysiology of lymphoma, amyloidosis, and factor X deficiency in a patient with preexisting angiodysplasias leading to refractory gastrointestinal bleeding. To our knowledge, there are only 3 reported cases of concomitant amyloidosis and angiodysplasia in the colon, and none involving the small bowel.

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Systemic Amyloidosis due to Low-Grade Lymphoma

Cited by 9 publications

References 45 publications

Marked progress in AL amyloidosis survival: a 40-year longitudinal natural history study

Marked progress in AL amyloidosis survival: a 40-year longitudinal natural history study

Constrictive Pericarditis Revealing Rare Case of ALH Amyloidosis With Underlying Lymphoplasmacytic Lymphoma (Waldenstrom Macroglobulinemia)

The 13-year bleed: Exuberant amyloid angiopathies, angiodysplasias, and acquired coagulopathies of the gut

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