Clinical Characteristics, Treatment Regimens, and Survival in Elderly Patients with AL Amyloidosis

Acevedo, Jose; Doros, Gheorghe; Szalat, Raphaël; Sanchorawala, Vaishali

doi:10.1016/j.clml.2021.01.021

Cited by 5 publications

(5 citation statements)

References 7 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Noticeably, no LCDD+AL patient J o u r n a l P r e -p r o o f 10 had lymphoma or Waldenström's macroglobulinemia which are present in ≈5% of patients with pure LCDD 8 or AL. The overall survival of our LCDD+AL patients was poor; median patient survival was 2.7 years, shorter than that reported for elderly pure AL patients (3.4 years) 24 and for general AL population (3.7 years) 25 . This could be multifactorial (advanced age, presence of co-morbidities, frequent high tumor burden, high incidence of ESKD, lack of treatment with bortezomib-based regimens in 60%).…”

Section: Discussioncontrasting

confidence: 69%

“…Other notable differences between our patients with LCDD+AL (without LCCN) and previously reported patients with pure AL or pure LCDD include higher frequency of females and microhematuria, lower frequency of other organ involvement (compared to AL), lower frequency of nodular glomerulosclerosis (compared to LCDD), and higher incidence of vascular-restricted AL deposits (compared to AL)(Table 4). 8,20,23,24,[26][27][28][29] Pathologically, LCDD+AL was more likely to be associated with λ than κ LC (λ:κ≈3:2).…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

The characteristics of patients with kidney light chain deposition disease concurrent with light chain amyloidosis

Said

Rocha

Valeri

et al. 2022

Kidney International

View full text Add to dashboard Cite

Section: Discussioncontrasting

confidence: 69%

Section: Discussionmentioning

confidence: 99%

The characteristics of patients with kidney light chain deposition disease concurrent with light chain amyloidosis

Said

Rocha

Valeri

et al. 2022

Kidney International

View full text Add to dashboard Cite

“…In this study, we detected significant improvements in survival over time for subgroups based on cardiac involvement and receipt of HDM/SCT, but not for patients with age at diagnosis ≥70 years. Older individuals with AL amyloidosis face distinct clinical challenges [ 41 , 42 ]. Adverse effects from multiorgan dysfunction in the presence of comorbidities, coupled with poor tolerance of therapies, are magnified in this subgroup.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2021

Self Cite

View full text Add to dashboard Cite

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.

show abstract

“…In general, the overall prognosis of oral amyloidosis is good, unless it is associated with systemic involvement, which can lead to a poor prognosis, with a median survival of 2 years. In particular, multiple myeloma combined with amyloidosis accounts for 6–15% of all cases [ 26 ]. These have variable prognosis, based on the multiple myeloma status [ 26 ].…”

Section: Discussionmentioning

confidence: 99%

“…In particular, multiple myeloma combined with amyloidosis accounts for 6–15% of all cases [ 26 ]. These have variable prognosis, based on the multiple myeloma status [ 26 ]. Other systemic conditions that may be associated with amyloidosis include tuberculosis, rheumatoid arthritis, Crohn’s disease, and Hodgkin’s lymphoma.…”

Section: Discussionmentioning

confidence: 99%

A 65-Year-Old Woman with an Enlarged Tongue Due to Amyloidosis

et al. 2022

View full text Add to dashboard Cite

Patient: Female, 65-year-old Final Diagnosis: Amyloidosis Symptoms: Enlarged tongue Medication:— Clinical Procedure: Intralesional steroid injection Specialty: Dentistry • General and Internal Medicine • Pathology • Rheumatology Objective: Unusual clinical course Background: Amyloidosis is a clinical condition characterized by the extracellular deposition of insoluble, abnormal amyloid fibrils in various body tissues. It is generally categorized into 2 forms – localized and systemic – with a wide range of signs and symptoms. This case report discusses the localized amyloidosis involvement of the oral cavity and its treatment. Case Report: A 65-year-old woman presented to the oral medicine clinic reporting painless tongue enlargement, which has been slowly progressing over several years, leading to difficulty in tongue movement, eating, and swallowing. Extra-oral examination showed a prominent lower lip with rubbery consistency. Intra-oral examination revealed a significantly enlarged tongue almost filling the whole oral cavity with dental indentations evident on all tongue surfaces and multiple, deep ulcerative craters of various sizes ranging from 2 to 5 mm in diameter. Histopathological examination under light microscope using hematoxylin and eosinophil and Congo red stain were diagnostic for amyloidosis. Further investigation with the Rheumatology Department, including renal and liver function tests, as well as echocardiography, were conducted and ruled out systemic involvement of other body organs. The patient was treated with weekly intra-lesional triamcinolone injections, with significant improvement. Conclusions: We report a rare case of localized amyloidosis presenting as macroglossia. Although the most effective management in tongue amyloidosis is surgical resection, conservative management in cases of localized oral amyloidosis presenting as macroglossia with weekly intra-lesional triamcinolone injections can be an effective approach, providing patients with better quality of life. Future studies exploring treatment modalities for similar cases with limited oral involvement are warranted.

show abstract

Clinical Characteristics, Treatment Regimens, and Survival in Elderly Patients with AL Amyloidosis

Cited by 5 publications

References 7 publications

The characteristics of patients with kidney light chain deposition disease concurrent with light chain amyloidosis

The characteristics of patients with kidney light chain deposition disease concurrent with light chain amyloidosis

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

A 65-Year-Old Woman with an Enlarged Tongue Due to Amyloidosis

Contact Info

Product

Resources

About