Primary systemic amyloidosis: multivariate analysis for prognostic factors in 168 cases

Abstract

One hundred sixty-eight patients with primary systemic amyloidosis (AL) were identified. Median survival after diagnosis was 12 months and ranged from 4 months for patients presenting with congestive heart failure to 50 months for those presenting with peripheral neuropathy only. Utilizing the proportional-hazards model in a stepwise multivariate fashion to evaluate the simultaneous influence of putative risk factors as of diagnosis revealed that congestive heart failure, urine light chain, hepatomegaly, and multiple myeloma were the major factors adversely affecting survival during the first year after diagnosis. Serum creatinine, multiple myeloma, orthostatic hypotension, and monoclonal serum protein were the most important variables adversely affecting survival for patients surviving 1 year. These models were used to categorize patients according to the variables in the models into low-, moderate-, and high-risk groups for the first year after diagnosis and separately for subsequent years. The influence of these variables on survival is important in stratification of patients randomized to prospective clinical trials.

Non‐invasive predictors of survival in cardiac amyloidosis

“…transthyretin or apolipoprotein AI, and may involve various organs [1]. Heart involvement is observed in about 50% of patients with light‐chain amyloidosis [2] and in some specific mutations in transthyretin amyloidosis and is by far the most relevant factor for poor prognosis of these patients [3–6]. Median survival in patients with symptoms of congestive heart failure is about six months [7].…”

Serum Amyloid A and P Protein Levels are Lowered by Dextran Sulfate Cellulose Low‐Density Lipoprotein Apheresis

“…This suggests that SAA mightbe associated with the formation of atheromatous lesions. Moreover, SAA hasan influence on the metabolism of high‐density lipoprotein HDL ( 21). On the other hand, SAP binds to all forms of amyloid fibril, is universallypresent in amyloid deposits ( 22), and is also found inatheromatous lesions ( 12).…”

“…No dramatic specific treatment has been reported for patients with primary or secondaryamyloidosis. However, a chelating agent for calcium ions experimentally couldsolubilize and remove amyloid proteins from amyloid deposits ( 21.) The use of polysulfone dialyzers or extracorporeal adsorbents may reducethe risk of β2‐microglobulin amyloidosis in chronic hemodialysispatients.…”

Outcome of patients with systemic light chain amyloidosis with concurrent renal and cardiac involvement

“…The accumulation of amyloidogenic protein can lead to rapid death if left untreated . The outcome of AL is heterogeneous, which depends on the amount of amyloidogenic protein, clonal plasma cells in the bone marrow, and the extent and the number of organs involved .…”

“…Studies have demonstrated that eGFR impairment and the amount of proteinuria predict the probability of progression to renal failure and response to therapy . Likewise, cardiac involvement and the extent of cardiac dysfunction with AL are independent prognostic determinants of survival . Dispenzieri and colleagues have described a cardiac staging system that utilizes cardiac biomarkers such as serum troponin T (cTnT) and N‐terminal pro‐brain natriuretic peptide (NT‐proBNP), and showed them to be strong predictors of overall survival for patients with AL .…”