The Systemic Amyloidoses

Falk, Rodney H.; Comenzo, Raymond L.; Skinner, Martha

doi:10.1056/nejm199709253371306

Cited by 1,154 publications

(961 citation statements)

References 77 publications

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“…The prognosis of AL amyloidosis patients were related with treatment option (chemotherapy or not), number of involved organs, pattern of involvement (heart and liver), and plasma cell burden. Cardiac amyloidosis is a major factor for prognosis in the other published results [15][16][17]. In our analysis, hematologic response, number of involved organs, and ECOG performance status were confirmed as independent prognostic factor in multivariate analysis.…”

Section: Introductionsupporting

confidence: 70%

Clinical features and treatment outcome of primary systemic light‐chain amyloidosis in Korea: Results of multicenter analysis

et al. 2012

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Primary systemic light-chain (AL) amyloidosis a disorder characterized by accumulation of monoclonal light chains as aggregated amyloid fibrils in tissues of multiple organs to cause organ dysfunction and death (Kyle and Gertz, Semin Hematol 1995;32:45-59; Merlini and Bellotti, N Engl J Med 2003;349:583-596). Although there are quite a number of data regarding clinical features and treatment outcomes of AL amyloidosis, most of them are from western countries except for a couple of reports from Japan (Kyle and Gertz,

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Section: Introductionsupporting

confidence: 70%

Clinical features and treatment outcome of primary systemic light‐chain amyloidosis in Korea: Results of multicenter analysis

et al. 2012

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“…Disease severity ranges from asymptomatic to severe, lifethreatening [4,7,25]. Beyond the initial symptoms like fatigue and weight loss, organs such as heart [26], kidney [27], gastrointestinal, liver, spleen [28] and nervous system (central and peripheral) [29] may involved with severe consequences.…”

Section: Discussionmentioning

confidence: 99%

Localized Tongue Amyloidosis in a Patient with Neurofibromatosis Type II

Andreadis¹,

Poulopoulos²,

Papadopoulos³

et al. 2011

Head and Neck Pathol

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Background Localized Amyloidosis (AL) may rarely involve oral mucosa. This is the first known reported case describing the development of tongue AL in a 30-year-old patient with Neurofibromatosis (NF) type-2. Case A female patient presented with a painless, well-circumscribed nodule of the tongue. Her medical history included NF type-2 with chromosome-22 abnormal karyotype (mosaicism), multiple intracranial and spinal meningiomas/ schwannomas and unilateral blindness/deafness. The biopsy of the excised lesion of the tongue revealed subepithelial accumulation of an amorphous, nodular, fibrillar material positive for Congo red. Blood examination showed increased Thyroxine-T4 due to thyroid multinodular colloid goiter, but excluded any other hematological/immunological disorder or organ dysfunction. No recurrence was observed after a sixmonth follow-up. Conclusion This case highlights the possibility of oral manifestations as the only sign of AL and reveals the unexpected co-existence of AL and NF 2, for the first time.

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“…This suggests that amyloid deposition may be a potential complication of morbid obesity. Secondary amyloidosis leads to proteinuria and microalbuminuria [43], which are urinary features seen in morbid obesity [44,45]. Histological studies are scarce, particularly in morbid obesity where kidney biopsy is technically difficult.…”

Section: Discussionmentioning

confidence: 99%

Serum amyloid A: production by human white adipocyte and regulation by obesity and nutrition

et al. 2005

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Aims/hypothesis: The acute-phase proteins, serum amyloid As (SAA), are precursors of amyloid A, involved in the pathogenesis of AA amyloidosis. This work started with the characterisation of systemic AA amyloidosis concurrent with SAA overexpression in the subcutaneous white adipose tissue (sWAT) of an obese patient with a leptin receptor deficiency. In the present study a series of histopathological, cellular and gene expression studies was performed to assess the importance of SAA in common obesity and its possible production by mature adipocytes. Materials and methods: Gene expression profiling was performed in the sWAT of two extremely obese patients with a leptin receptor deficiency. Levels of the mRNAs of the different SAA isoforms were quantified in sWAT cellular fractions from lean subjects and from obese subjects before and after a very-low-calorie diet. These values were subsequently compared with serum levels of SAA in these individuals. In addition, histopathological analyses of sWAT were performed in lean and obese subjects. Results: In sWAT, the expression of SAA is more than 20-fold higher in mature adipocytes than in the cells of the stroma vascular fraction (p<0.01). Levels of SAA mRNA expression and circulating levels of the protein are sixfold (p<0.001) and 3.5-fold (p<0.01) higher in obese subjects than in lean subjects, respectively. In lean subjects, 5% of adipocytes are immunoreactive for SAA, whereas the corresponding value is greater than 20% in obese subjects. Caloric restriction results in decreases of 45-75% in levels of the transcripts for the SAA isoforms and in circulating levels of the protein. Conclusions/interpretation: The results of the present study indicate that SAA is expressed by sWAT, and its production at this site is regulated by nutritional status. If amyloidosis is seen in the context of obesity, it is possible that production of SAA by adipocytes could be a contributory factor.

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The Systemic Amyloidoses

Cited by 1,154 publications

References 77 publications

Clinical features and treatment outcome of primary systemic light‐chain amyloidosis in Korea: Results of multicenter analysis

Clinical features and treatment outcome of primary systemic light‐chain amyloidosis in Korea: Results of multicenter analysis

Localized Tongue Amyloidosis in a Patient with Neurofibromatosis Type II

Serum amyloid A: production by human white adipocyte and regulation by obesity and nutrition

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