scholarly journals Development and validation of a survival staging system incorporating BNP in patients with light chain amyloidosis

Blood ◽  
2019 ◽  
Vol 133 (3) ◽  
pp. 215-223 ◽  
Author(s):  
Brian Lilleness ◽  
Frederick L. Ruberg ◽  
Roberta Mussinelli ◽  
Gheorghe Doros ◽  
Vaishali Sanchorawala
Keyword(s):  
Brain Natriuretic Peptide ◽  
Natriuretic Peptide ◽  
Light Chain ◽  
Cardiac Involvement ◽  
Staging System ◽  
Stage I ◽  
Stage Iiib ◽  
Stage Ii ◽  
Al Amyloidosis ◽  
Derivation Cohort

Abstract Immunoglobulin light chain amyloidosis (AL amyloidosis) is caused by misfolded light chains that form soluble toxic aggregates that deposit in tissues and organs, leading to organ dysfunction. The leading determinant of survival is cardiac involvement. Current staging systems use N-terminal pro-brain natriuretic peptide (NT-proBNP) and cardiac troponins T and I (TnT and TnI) for prognostication, but many centers do not offer NT-proBNP. We sought to derive a new staging system using brain natriuretic peptide (BNP) that would correlate with the Mayo 2004 staging system and be predictive for survival in AL amyloidosis. Two cohorts of patients were created: a derivation cohort of 249 consecutive patients who had BNP, NT-proBNP, and TnI drawn simultaneously to create the staging system and a complementary cohort of 592 patients with 10 years of follow-up to determine survival. In the derivation cohort, we found that a BNP threshold of more than 81 pg/mL best associated with Mayo 2004 stage and also best identified cardiac involvement. Three stages were developed based on a BNP higher than 81 pg/mL and a TnI higher than 0.1 ng/mL and compared with Mayo 2004 with high concordance (κ = 0.854). In the complementary cohort, 25% of patients had stage I, 44% had stage II, 15% had stage III, and 16% had stage IIIb disease with a median survival not reached in stage I, 9.4 years in stage II, 4.3 years in stage III, and 1 year in stage IIIb. This new Boston University biomarker scoring system will allow centers without access to NT-proBNP the ability to appropriately stage patients with AL amyloidosis. This trial was registered at www.clinicaltrials.gov as #NCT00898235.

scholarly journals The Clinical Features of Multiple Myeloma with AL Amyloidosis

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 10-10
Author(s):  
Junichiro Nashimoto ◽  
Moe Nomura ◽  
Hirofumi Irita ◽  
Kota Sato ◽  
Kanji Miyazaki ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Clinical Features ◽  
Light Chain ◽  
Mayo Clinic ◽  
Plasma Cells ◽  
Staging System ◽  
Stage I ◽  
P Value ◽  
Al Amyloidosis ◽  
Hepatic Amyloidosis

Background Multiple myeloma and AL amyloidosis are both caused by the clonal proliferation of the abnormal plasma cells. Although, the difference of the genetic features of multiple myeloma and AL amyloidosis has been reported, we see some patients present with both cases. We retrospectively investigated the clinical features of patients diagnosed with multiple myeloma and AL amyloidosis. Methods We reviewed medical records of patients who were diagnosed with multiple myeloma and AL amyloidosis before initiating treatment during January 2009 to November 2019 in our institution. We excluded patients who did not reach 10% of the plasma cells in the bone marrow. Patients diagnosed with light chain deposition disease were excluded. Treatment regimens were at the discretion of the treating physician. Results Forty-two patients were diagnosed with multiple myeloma and AL amyloidosis. The median follow-up time since diagnosis was 20 months [0-89]. The median age was 63-year-old [43-85]. There was no difference between the sex (male: female=1:1). Twenty-nine (69.0%) patients had lambda type of light chain. Patients with ISS stage I, II, and III were 13(31.7%), 21(51.2%), and 7(17.1%). Patients with R-ISS stage I, II, and III were 4(10.3%), 30(76.9%), and 5(12.8%). Patients with Revised Mayo Clinic AL amyloidosis Staging System 1, 2, 3, and 4 were 3(10.0%), 4(13.3%), 8(26.7%), and 15(50.0%). Twelve (35.3%), 2 (6.9%), 1 (3.4%) and 1 (4.0%) patients had t(11;14), t(4;14), t(14;16) and del(17p) by FISH analysis, respectively. Fourteen (33.3%), 16(38.1%), and 8(19.0%) patients were diagnosed with cardiac, renal, and hepatic amyloidosis, respectively. Thirty-five (83.3%) patients received Bortezomib containing regimen for the initial treatment (e.g., Bortezomib+Dexamethasone(7.1%), Cyclophosphamide+Bortezomib+Dexamethasone(23.8%), Bortezomib+Melphalan+Dexamethasone(7.1%), Bortezomib+Melphalan+Prednisolone(9.5%), Bortezomib+Lenalidomide+Dexamethasone(35.7%)). Thirteen (31.0%) patients underwent autologous stem cell transplantation with high dose melphalan. Median PFS was 25 months and the median OS was 82 months. There were no significant differences in OS between the I-SSS, R-ISSS, and Revised Mayo Clinic AL amyloidosis Staging System groups. Patients diagnosed with cardiac or hepatic amyloidosis had significantly worse outcome. The median OS diagnosed with and without cardiac amyloidosis were 14 and 28 months (p value = 0.034), and the median OS diagnosed with and without hepatic amyloidosis were 8 and 24 months (p value = 0.012). Conclusion Multiple myeloma with AL amyloidosis, especially cardiac or hepatic amyloidosis, has a poor prognosis even if treated with novel agents. Searching for the optimal treatment for these patient groups remains to be an issue. Disclosures Nashimoto: Janssen: Speakers Bureau; Celgene: Speakers Bureau. Tsukada:Takeda pharmaceutical co: Speakers Bureau. Ishida:Ono pharmaceutical co: Speakers Bureau; Takeda pharmaceutical co: Speakers Bureau; Janssen: Speakers Bureau; Celgene: Speakers Bureau. Suzuki:Bristol-Myers Squibb, Celgene and Amgen: Research Funding; Takeda, Amgen, Janssen and Celgene: Consultancy; Takeda, Celgene, ONO, Amgen, Novartis, Sanofi, Bristol-Myers Squibb, AbbVie and Janssen: Honoraria.

scholarly journals The Impact of Longitudinal Strain on Haematological and Cardiac Response and Survival in Patients with Systemic AL Amyloidosis

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 40-40
Author(s):  
Oliver C Cohen ◽  
Andreia Ismael ◽  
Richa Manwani ◽  
Sriram Ravichandran ◽  
Steven Law ◽  
...  
Keyword(s):  
Longitudinal Strain ◽  
Cardiac Involvement ◽  
Troponin T ◽  
Staging System ◽  
Stage I ◽  
Cardiac Response ◽  
Al Amyloidosis ◽  
International Consensus ◽  
The Uk ◽  
The Impact

Background: Cardiac involvement is the major determinant of prognosis in systemic AL amyloidosis. The extent is assessed by cardiac biomarker-based staging system using N-terminal pro-brain natriuretic peptide (NT-proBNP) and Troponin T. Longitudinal strain evaluates the global and regional function of the left ventricle (LV) and may be preferable to both LV ejection fraction and NT-proBNP, which is limited by its sensitivity to changes in fluid balance, in determining prognosis. This is the first report of a large cohort of uniformly treated prospectively followed patients assessing the utility of changes in longitudinal function by 2-D strain (LS%), impairment of which is a hallmark of amyloidosis. Methods: 915 newly diagnosed patients seen at the UK National Amyloidosis Centre (February 2010 - August 2017) were studied. All patients underwent comprehensive assessments including echo-cardiogram at baseline and each follow up visit. Results: 628/915 (68.6%) patients had cardiac involvement. Mayo stage I, II, IIIa and IIIb in 144 (15.7%), 302 (33.0%) 344 (37.6%) and 125 (13.7%) respectively. Impairment of LS% correlated significantly with increasing Mayo stage (p<0.0001 between LS% for each Mayo stage). At 12 months, only patients with complete haematological responses (CR) had significant improvement in LS% (overall p=0.04; regional baso-lateral p=0.007, and baso-septal p=0.007). The median overall survival (OS) of the whole cohort was 61 months; survival of Mayo stage I and II patients was not reached whilst OS in Mayo stage IIIa and IIIb patients was 30 and 4 months respectively. Patients with cardiac involvement were stratified into 3 baseline LS% groups (≤17%; 10.3-16.9%; and ≥10.2%) with poor baseline LS% being associated with shorter OS (p<0.0001). These groups predicted survival independently of Mayo stage. OS was superior in patients who achieved a minimum absolute improvement in LS% of 1.5% when analysed at either 12 (not reached vs. 72 months, p=0.008) and 24 (not reached vs. 80 months, p<0.0001) months from diagnosis. Patients achieving a LS% response (1.5%) improvement survived longer than those achieving a traditional cardiac response alone or no cardiac response at both 12 and 24 months (p<0.0001). Conclusion: Longitudinal strain is an informative functional marker that is independent of Mayo staging in predicting outcomes in patients with cardiac AL amyloidosis which can be incorporated in prognostic staging for these patients. Improvement in LS% was observed in patients who achieved a CR, and a value of 1.5% was associated with superior outcomes over and above achieving a cardiac response by international consensus criteria. An absolute improvement in LS% should be considered a criterion for cardiac response in AL amyloidosis. Disclosures Wechalekar: Caelum: Other: Advisory; Janssen: Honoraria, Other: Advisory; Takeda: Honoraria, Other: Travel; Celgene: Honoraria.

A Novel Prognostic Staging System for Light Chain Amyloidosis (AL) Incorporating Markers of Plasma Cell Burden and Organ Involvement.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 2797-2797 ◽  
Author(s):  
Shaji Kumar ◽  
Angela Dispenzieri ◽  
Martha Q. Lacy ◽  
Suzzane Hayman ◽  
Francis Buadi ◽  
...  
Keyword(s):  
Risk Stratification ◽  
Plasma Cell ◽  
Light Chain ◽  
Cardiac Involvement ◽  
Troponin T ◽  
Prognostic Score ◽  
Staging System ◽  
Cardiac Biomarkers ◽  
Al Amyloidosis ◽  
The Impact

Abstract Abstract 2797 Poster Board II-773 Background: Primary systemic amyloidosis (AL) is characterized by deposition of light chain derived amyloid fibrils in multiple organs leading to varying degree of dysfunction. Cardiac involvement is a common feature of AL, and when significant is highly predictive of poor outcome. The currently used prognostic classification is based on cardiac biomarkers troponin-T (cTnT) and N-terminal pro B-type Natriuretic peptide (NT-ProBNP) and is effective at predicting outcome in patients with AL. However, this is driven primarily by the degree of cardiac involvement and does not take into account the degree of plasma cell burden and its impact on the disease course. It is possible that some of the heterogeneity in outcome can be explained by the plasma cell characteristics rather than the degree of the end organ damage. Methods: We examined the baseline clinical and laboratory data from 2119 patients with AL who were seen at our institution with in 90 days of their diagnosis. The data were collected from a prospectively maintained data base, and additional testing was done for free light chain levels (FLC) on stored sera from patients seen before the routine introduction of FLC testing. Cox proportional hazards analysis was performed to estimate the prognostic values of different variables. Results: We first examined the impact of plasma cell clone related characteristics namely, difference between involved and uninvolved FLC (FLC-Diff), bone marrow plasma cell (BMPC) %, plasma cell labeling index (PCLI), beta 2 microglobulin (B2M), and presence of circulating plasma cells, on overall survival (OS) from diagnosis. All variables were dichotomized into high and low based on their median value (FLC-diff: 20 mg/dL, BMPC: 10%, PCLI as a continuous variable, B2M: 3 mg/dL and circulating cells: absent or present). While all were found to be prognostic for OS in univariate analysis; in a multivariate analysis incorporating a stepwise selection only B2M and FLC-diff were significant. We then used these two variables along with the cTnT and NT-ProBNP that are used in the current model to develop a staging system. The median values for all four variables were used for developing the scores. Patients were assigned a score of 1 for presence of each characteristic (FLC difference > 20 mg/dL, troponin-T > 0.02, NT-ProBNP > 1000, and B2M > 3) or 0 if the value was at or below the cutoff. The scores were added to obtain a composite prognostic score that grouped the 370 patients (who had all the variables available for analysis) into 5 groups with very different OS (4.4, 9.3, 24.4, 43, and not reached for stages 4, 3, 2, 1, and 0 respectively; Figure A). However, since the NT-ProBNP did not have independent impact on survival in a multivariable model incorporating all the four variables, we also examined a system that only included FLC-diff, cTnT and B2M. This system again allowed grouping of patients (n=450 with all three variables available) into four distinct groups with divergent outcome with OS of 4.6, 10.5, 36.8, and not reached for stages 3, 2, 1, and 0 respectively; (Figure B). Conclusion: Incorporation of plasma cell related measurements into the existing staging system using cardiac biomarkers allows better risk stratification of patients with AL amyloidosis. The system using FLC, B2M and troponin has the advantage of easily available laboratory values and can be widely adopted. Addition of NT-ProBNP into the system allows better refinement of the intermediate risk groups. This system will allow upfront risk stratification and development of risk-adapted therapies for patients with AL. Disclosures: Gertz: Genzyme: Research Funding.

scholarly journals N-Terminal Pro-Brain Natriuretic Peptide (NT-proBNP) Is Associated with Both Left Ventricular Diastolic Dysfunction and Myeloma-Related Renal Insufficiency and Robustly Predicts Mortality in Patients with Symptomatic Multiple Myeloma

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 4478-4478
Author(s):  
Yoshiaki Abe ◽  
Tetsuya Kobayashi ◽  
Kentaro Narita ◽  
Hiroki Kobayashi ◽  
Akihiro Kitadate ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Renal Insufficiency ◽  
Brain Natriuretic Peptide ◽  
Natriuretic Peptide ◽  
Light Chain ◽  
Prognostic Value ◽  
Left Ventricular ◽  
Al Amyloidosis ◽  
Newly Diagnosed ◽  
Renal Functions

Abstract Background: AlthoughN-terminal pro-brain natriuretic peptide (NT-proBNP) is an established biomarker as a prognostic predictor in patients with light-chain (AL) amyloidosis, its prognostic value and association with cardiac or renal functions remain unclear in patients with symptomatic multiple myeloma (MM). The aims of this study included to validate the prognostic significance of the baseline NT-proBNP levels comparing with the brain natriuretic peptide (BNP) levels and to identify clinical factors and mechanisms which are reflected by NT-proBNP and more directly affect mortality in newly diagnosed, symptomatic MM. Methods: We retrospectively analyzed prognostic relevance of NT-proBNP in comparison with brain natriuretic peptide (BNP) and its association with cardiac functions on echocardiography or renal functions in 153 consecutive patients with newly diagnosed, symptomatic MM who were diagnosed and treated with chemotherapy between April 2008 to March 2018 at Kameda Medical Center, Kamogawa, Japan. We included only patients who had been treated with novel agents. Patients with pathologically proven organ involvement with light-chain (AL) amyloidosis were excluded from the analysis. The presence of LV diastolic dysfunction (LVDD) was determined individually according to the 2016 American Society of Echocardiography and European Association of Cardiovascular Imaging guidelines. Results: Median age of the patients was 74.2 years [interquartile range (IQR): 66.7-80.8 years]. The median observation period was 26.4 months (IQR: 10.8-49.1 months). Receiver operating characteristic(ROC) curves for BNP and NT-proBNP predicting the highest risk of death within five years were shown in Figure 1A and 1B, respectively. According to the ROC analysis, the optimal cutoff of NT-proBNP was determined as 341.0 pg/ml. The comparison of area under curve (AUC) between BNP and NT-proBNP was shown in Figure 1C. The AUC was significantly greater for NT-proBNP than for BNP (0.818 vs. 0.731; P=0.024).Subsequently, we divided patients into two patient groups with lower (n=83) and higher (n=68) NT-proBNP according to the cutoff. Patients with higher NT-proBNP were significantly older, had poorer performance status, Instrumental Activity of Daily Living and Charlson Comorbidity Index scores, and included more patients with higher disease stages. Notably, patients with higher NT-proBNP included more patients with LVDD than patients with lower NT-proBNP (71.4% vs. 33.3%, respectively; P<0.001, Figure 2A), and all diastolic function-related parameters showed associations with NT-proBNP, although the difference in septal e' did not reach statistical significance (Figure 2B-E). However, there was no significant difference in left ventricular ejection fraction between patients with lower and higher NT-proBNP (Figure 2F). We also investigated the association between NT-proBNP and myeloma-related renal insufficiency shown in Figure 3. Patients with higher NT-proBNP included more patients with renal insufficiency (58.8% vs. 15.3%, respectively; P<0.001) or light-chain cast nephropathy (41.2% vs. 8.2%, respectively; P<0.001) with their involved free-light chain and corrected calcium levels significantly higher than patients with lower NT-proBNP (Figure 3A-D). Patients with higher NT-proBNP showed significantly shorter overall survival (OS) than those with lower NT-proBNP (median: 33.6 months and not reached, respectively; P<0.001, Figure 4). NT-proBNP levels discriminated patients with significantly different survivals even in both younger (<75 years) and older (≥75 years)patients or in both patients with and without decreased renal functions (estimated glomerular filtration rate ≥ and <50 ml/min/1.73m2).Furthermore, NT-proBNP retained its significant prognostic value for OS on multivariate analysis with the highest hazard ratio (HR) (HR; 7.66, 95% confidence interval; 3.52-16.68, P<0.001) (Table 1). Conclusions: Our findings revealed that the NT-proBNP levels were associated with both LVDD as a host risk factor and myeloma-related renal insufficiency resulting from aggressive disease nature and consequently provided a robustly predictive information for OS in patients with symptomatic MM who did not harbor concomitant AL amyloidosis. Further studies should explore the synergistic prognostic potential of NT-proBNP. Disclosures No relevant conflicts of interest to declare.

The Utility of High Sensitivity Cardiac Troponin Among Patients with Immunoglobulin Light Chain Amyloidosis

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 2887-2887 ◽  
Author(s):  
Angela Dispenzieri ◽  
Morie A Gertz ◽  
Amy K Saenger ◽  
Martha Grogan ◽  
Shaji Kumar ◽  
...  
Keyword(s):  
Light Chain ◽  
Cardiac Involvement ◽  
High Sensitivity ◽  
Staging System ◽  
Cardiac Response ◽  
Al Amyloidosis ◽  
Immunoglobulin Light Chain ◽  
Light Chain Amyloidosis ◽  
Staging Systems ◽  
Immunoglobulin Light Chain Amyloidosis

Abstract Abstract 2887 Introduction: Cardiac involvement is the major cause of death in patients with immunoglobulin light chain amyloidosis (AL). Detection of cardiac involvement and risk stratification has been facilitated by cardiac biomarkers like troponin T (cTnT) and N-terminal brain natriuretic peptide (NT-proBNP). A novel high sensitivity cTnT (hs-cTnT) assay has been developed, and we evaluated its diagnostic use with three questions in mind: 1) How do the cTnT and hs-cTnT perform in the AL amyloid staging system? 2) Does higher sensitivity add significant additional value in terms of prognosticating outcomes for patients with AL amyloidosis? 3) Can the current AL amyloidosis staging system be further improved upon? Methods: Stored serum samples (-20°C) from 224 pts with AL were analyzed for concentrations of hsTnT, TnT, and NT-proBNP on the E170 Modular analyzer (Roche Diagnostics, Penzberg, Germany). 99th percentile reference limits were <0.014 and <0.010 mcg/L for hsTnT and TnT, respectively. Results: Median values for hsTnT, TnT, and NT-proBNP were 38 ng/L (range 0–075.4), 0.017 mcg/L (<0.0–0.904), and 1230 ng/L (0–32, 226), respectively. The correlation coefficient between hsTnT and TnT was 0.972. Those classified by echocardiographic parameters as having (n=143) or not having (n=81) cardiac involvement had TnT concentrations of 0.04 and 0.01 mcg/L and hsTnT levels of 52.2 and 15.6 ng/L, respectively. The direct numeric result from the hs-cTnT result CANNOT merely be substituted for a cTnT result in the Mayo AL staging system since 14% of patients would be misclassified. The performance of the receiver operation curve derived hs-cTnT cut-point of 54 ng/L is a slight improvement over the direct substitution of 35 ng/L if replacement of one assay for another is required. An alternate staging option using hs-cTnT alone—using the two thresholds14 ng/L and 54 ng/L (figure)—performs as well as either the original Mayo AL staging system or a derivative system incorporating hs-cTnT with respective relative risks of death (95%CI) of 3.6 (2.3, 5.7), 3.8 (2.5, 5.9), and 3.3 (2.2, .50). On multivariate analysis, our newly described alternate 3 level, hs-cTnT alone staging system is independent of other factors including period of diagnosis, type of therapy, and NT-proBNP value, the last of which dropped out of the model. Alternate models using NT-proBNP and cTnT were explored, but none performed better than the original staging system or the new hs-cTnT system. Conclusion: The direct numeric result from the hs-cTnT result cannot merely be substituted for a cTnT result in the Mayo amyloid staging system. Consideration could be made for AL staging systems using hs-cTnT alone and relegating the NT-proBNP for measuring cardiac response. Disclosures: Jaffe: Roche: Consultancy.

Front-line daratumumab in newly diagnosed AL amyloidosis patients.

2020 ◽  
Vol 38 (15_suppl) ◽  
pp. e20569-e20569
Author(s):  
Vanessa Elaine Kennedy ◽  
Nina Shah ◽  
Jeffrey Lee Wolf ◽  
Thomas G. Martin ◽  
Sandy Wai Kuan Wong
Keyword(s):  
Median Time ◽  
Light Chain ◽  
Stage I ◽  
Stage Ii ◽  
Stage Iii ◽  
Al Amyloidosis ◽  
Newly Diagnosed ◽  
First Line ◽  
Best Response ◽  
First Response

e20569 Background: Light chain amyloidosis (AL) is a plasma cell dyscrasia characterized by organ dysfunction, morbidity, and early mortality. Treatment focuses on suppressing monoclonal light chain production as measured by hematologic response criteria (Comenzo et al., 2012). Daratumumab (DARA), a monoclonal CD38 antibody, has demonstrated efficacy in heavily pretreated AL patients (Kaufman et al., 2017). We report the outcomes of newly diagnosed AL patients treated with a DARA-based regimen. Methods: A single-center retrospective chart review with an IRB-approved protocol was performed on newly diagnosed AL patients treated with first line DARA-based regimens between 1/2018 – 12/2019. Results: Fourteen patients were evaluated. The median age was 69 years (45 – 87), 50% were male, and 50% produced monoclonal lambda light chains. All patients had cardiac and renal involvement. Per the 2004 Mayo staging system, 1/14 (7%) patients had stage I cardiac disease, 2/14 (14%) had stage II, and 11/14 (79%) had stage III, of which two were stage IIIb. Regarding renal staging (Palladini et al., 2014), 10/14 (71%) patients were stage I, 3/14 (21%) stage II, and 1/4 (7%) stage III. Most patients (11/14) received DARA in combination with bortezomib and dexamethasone, while one patient received DARA and dexamethasone (DEX). Two patients received DARA in combination with bortezomib, cyclophosphamide, and DEX. Patients received a median of 5 cycles of DARA (1 – 14). After a median follow-up of 4.4 months (1 – 19), all patients were still receiving DARA. DARA was well tolerated, with 4 patients (29%) experiencing grade 1 infusion reactions and no additional toxicities observed. Responses were rapid, with a median time of 11 days (7 – 61) to first response and 46 days (9 8– 168) to best response. First response was classified as partial response (PR) in 8/14 patients (57%), very good PR (VGPR) in 3/14 (21%), and complete response (CR) in 1/14 (7%). Best response included a PR in 3/14 (21%), VGPR in 7/14 (50%), and CR in 2/14 (14%). In most patients, responses deepened over time. Cardiac response was seen in 5/7 (71%) evaluable patients with median time to response of 39 days (21 – 263). Renal response was seen in 2/4 (50%) evaluable patients with median time to response of 152 days (94 – 194). Conclusions: First line use of DARA in AL amyloidosis produced rapid and deep hematologic and organ response, resulting in a 93% objective hematologic response rate. DARA was well-tolerated and efficacious even among patients with extensive organ involvement and cardiac dysfunction. Prospective studies of first line DARA are warranted.

scholarly journals Diagnostic approach to light-chain cardiac amyloidosis and its differential diagnosis

2018 ◽  
Vol 49 (1) ◽  
pp. 9-14
Author(s):  
Monika Adamska ◽  
Anna Komosa ◽  
Tatiana Mularek ◽  
Joanna Rupa-Matysek ◽  
Lidia Gil
Keyword(s):  
Differential Diagnosis ◽  
Light Chain ◽  
Cardiac Amyloidosis ◽  
Cardiac Involvement ◽  
Diagnosis And Treatment ◽  
Diagnostic Approach ◽  
Al Amyloidosis ◽  
Delay In Diagnosis ◽  
Light Chain Amyloidosis ◽  
Novel Approaches

AbstractCardiac amyloidosis is a rare and often-misdiagnosed disorder. Among other forms of deposits affecting the heart, immunoglobulin-derived light-chain amyloidosis (AL amyloidosis) is the most serious form of the disease. Delay in diagnosis and treatment may have a major impact on the prognosis and outcomes of patients. This review focuses on the presentation of the disorder and current novel approaches to the diagnosis of cardiac involvement in AL amyloidosis.

Clinical Staging for Sleep-Disordered Breathing

2002 ◽  
Vol 127 (1) ◽  
pp. 13-21 ◽  
Author(s):  
Michael Friedman ◽  
Hani Ibrahim ◽  
Lee Bass
Keyword(s):  
Body Mass Index ◽  
Success Rate ◽  
Body Mass ◽  
Sleep Disordered Breathing ◽  
Staging System ◽  
Stage I ◽  
Stage Ii ◽  
Stage Iii ◽  
Clinical Staging ◽  
Disordered Breathing

OBJECTIVE: The purpose of this study was to identify prognostic indicators that would lead to stratification of patients likely to have successful surgery for sleep-disordered breathing (SDB) versus those destined to fail. STUDY DESIGN: We retrospectively reviewed 134 patients to correlate palate position and tonsil size to the success of the UPPP as based on postoperative polysomnography results. Similar to our previously published data on the Friedman Score as a predictor of the presence and severity of SDB, the palate position was determined on physical examination of the oral cavity and was graded for each patient. This grade combined with tonsil size was used to stage the patients. Stage I was defined as having palate position 1 or 2 combined with tonsil size 3 or 4. Stage II was defined as having palate position 3 or 4 and tonsil size 3 or 4. Stage III patients had palate position 3 or 4 and tonsil size 0, 1, or 2. Any patient with body mass index of greater than 40 was placed in the stage III group. The results of uvulopalatopharyngoplasty (UPPP) were then graded as success or failure and success rates were compared by stage. SETTING: Academically affiliated tertiary care referral center. RESULTS: Stage I patients who underwent UPPP had a success rate of 80.6%, stage II patients had a success rate of 37.9%, and stage III patients had a success rate of 8.1%. CONCLUSION: A clinical staging system for SDB based on palate position, tonsil size, and body mass index is presented. It appears to be a valuable predictor of the success of UPPP. Additional studies and wider use of the staging system will ultimately define its role in the treatment of SDB.

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