scholarly journals Grade 3 myocardial uptake in 99mtechnetium-pyrophosphate scintigraphy in light chain cardiac amyloidosis

Author(s):  
Atsushi Okada ◽  
Emi Tateishi ◽  
Keiko Ohta-Ogo ◽  
Chisato Izumi

Abstract We report grade 3 myocardial uptake in 99mtechnetium-pyrophosphate (99mTc-PYP) scintigraphy in a case of light-chain cardiac amyloidosis (CA), which emphasizes the importance of screening for monoclonal protein even in typical 99mTc-PYP findings of transthyretin CA.

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 1960-1960 ◽  
Author(s):  
Stefan O Schonland ◽  
Tilmann Bochtler ◽  
Anthony D. Ho ◽  
Ute Hegenbart

Abstract Abstract 1960 Purpose: Severe cardiac involvement is known to confer a poor prognosis in patients with light chain (AL-) amyloidosis. These patients do not qualify for high-dose chemotherapy regimens. In a recent analysis we could show that melphalan/dexamethasone therapy was not able to overcome this poor prognosis (Dietrich et al, 2010). Since bortezomib is known to rapidly lower the involved light chain levels (iFLC), it appears a promising alternative regimen. There is only one prospective phase I/II trial in AL amyloidosis using single agent bortezomib (Reece et al., 2009). However, patients with advanced cardiac amyloidosis had been excluded. We therefore assessed the feasibility of bortezomib/dexamethasone (Bd) in patients with high-risk cardiac amyloidosis as defined by Mayo Clinic stage III (median overall survival (OS) 3.5 months (mo), Dispenzieri et al, 2004). Patients and Method: We retrospectively evaluated 38 consecutive patients with systemic AL amyloidosis, who were classified as Mayo Clinic stage III cardiac amyloidosis based on their elevated cardiac biomarkers NT-proBNP, TNT and hsTNT values (patient characteristics table 1). 14 patients were untreated. For the sake of tolerability Bd was administered twice weekly with a reduced bortezomib dosage of 4 × 1.0 mg/m2 and dexamethasone 8 × 8 mg in a 3 week schedule. Result: Median observation of living patients is 5 mo after start of Bd. Median OS is 9 mo (Figure 1a). 18 patients died; among them 11 (29%) during ongoing bd. Median OS of the first line therapy group was 7 mo versus 13 mo in the relapsed group (p=0.056, Figure 1b). 21 patients achieved a hematological remission (HR) after a median of 3 cycles (64% out of 33 evaluable patients, 2 CRs and 19 PRs), whereas 3 patients died during the first Bd cycle and further 2 just started with Bd. Hematologic toxicity grade 3 or 4 according to NCI criteria was observed in 9 patients (24%) (toxicities are listed in table 1). Apart from the 11 early deaths we observed non-hematologic toxicity grade 3 in 8 patients and grade 4 in 6 patients. In 3 patients, the bortezomib dose had to be reduced due to toxicity. Therapy was stopped before the planned 6 – 8 cycles in 11 patients, in 4 cases due to toxicity, in 5 cases due to lacking HR and in 2 cases due to patient‘s choice. 9 patients completed their therapy regularly in HR while therapy is still ongoing in 5 patients. It is planned to update these data in November 2010. Discussion: The goal of this study was to assess whether patients with severe cardiac amyloidosis as defined by Mayo Clinic stage III benefit from Bd therapy. As already known from other studies, we observed a fast reduction of iFLC within 3 months from 203 to 81 mg/l (of evaluable patients) in median. Overall, HR rate was 64%, which is comparable to 71% previously reported by Kastritis et al, 2010. However, toxicity and early death rate were considerable. Main problem of the newly diagnosed patients was the severity of cardiac involvement (median NTpro BNP 11.346 ng/l) leading to a high early mortality. Main problem of the pretreated patients was the lower HR rate of 52% versus 83% of the newly diagnosed patients. Though we observed substantial toxicities in spite of the reduced Bd dosages, the rather poor OS does not appear to be largely due to toxicity. Conclusion: Dose-reduced Bd might be an important treatment option for patients with severe cardiac AL amyloidosis Mayo Clinic stage III because of its high efficacy. However, the toxicity is still significant. Inpatient care for the first cycle of Bd might lead to a reduction of the early death rate. Disclosures: No relevant conflicts of interest to declare.


Author(s):  
Ahmad Masri ◽  
Syed Bukhari ◽  
Shahzad Ahmad ◽  
Ricardo Nieves ◽  
Yvonne S. Eisele ◽  
...  

Background: Technetium-99 m pyrophosphate protocols for transthyretin cardiac amyloidosis diagnosis have variably used 1- and 3-hour imaging time points. We investigated whether imaging at 1 hour with superior efficiency had comparable diagnostic accuracy as 3-hour imaging. Methods: This is a registry analysis of patients with suspected transthyretin cardiac amyloidosis referred for technetium-99 m pyrophosphate at a single tertiary center from June 2015 through January 2019. Patients underwent planar and single-photon emission computed tomography (SPECT) imaging at 1 and 3 hours. A positive Tc-99m pyrophosphate study was defined by the presence of diffuse myocardial tracer uptake on SPECT. For planar imaging, visual semiquantitative (grades 0-3, ≥2 considered positive) and quantitative heart to contralateral ratios (≥1.5 considered positive) were used. Results: Two hundred thirty-three patients (69% men; median age, 77 [69–83] years) underwent the study protocol. There were 60 (25.8%) patients with diffuse myocardial uptake, 1 (0.4%) with regional uptake, and 172 (73.8%) with no myocardial uptake. Results of SPECT were identical at 1 and 3 hours. Planar imaging at 1 hour had 98% sensitivity and 96% specificity. Planar grade 0 uptake or heart to contralateral ratio ≤1.2 and planar grade 3 uptake or heart to contralateral ratio ≥2.0 were always associated with negative and positive SPECT, respectively. For planar grades 1 and 2 uptake and heart to contralateral ratio 1.3 to 1.9, SPECT was needed to make a diagnosis. No patient with light-chain cardiac amyloidosis had positive SPECT. Conclusions: An efficient 1-hour technetium-99 m pyrophosphate protocol had comparable diagnostic performance to a 3-hour protocol.


2021 ◽  
Vol 8 (1) ◽  
Author(s):  
Chao Ren ◽  
Jingyun Ren ◽  
Zhuang Tian ◽  
Yanrong Du ◽  
Zhixin Hao ◽  
...  

Abstract Background 99mTc-PYP scintigraphy provides differential diagnosis of ATTR cardiomyopathy (ATTR-CM) from light chain cardiac amyloidosis and other myocardial disorders without biopsy. This study was aimed to assess the diagnostic feasibility and the operator reproducibility of 99mTc-PYP quantitative SPECT. Method Thirty-seven consecutive patients who underwent a 99mTc-PYP thorax planar scan followed by SPECT and CT scans to diagnose suspected ATTR-CM were enrolled. For the quantitative SPECT, phantom studies were initially performed to determine the image conversion factor (ICF) and partial volume correction (PVC) factor to recover 99mTc-PYP activity concentration in the myocardium for calculating the standardized uptake value (SUV) (unit: g/ml). SUVmax was compared among groups of ATTR-CM, AL cardiac amyloidosis, and other pathogens (others) and among categories of Perugini visual scores (grades 0–3). The intra- and inter-operator reproducibility of quantitative SPECT was verified, and the corresponded repeatability coefficient (RPC) was calculated. Results The ICF was 79,327 Bq/ml to convert count rate in pixel to 99mTc activity concentration. PVC factor as a function of the measured activity concentration ratio in the myocardium and blood-pool was [y = 1.424 × (1 − exp(− 0.759 × x)) + 0.104]. SUVmax of ATTR-CM (7.50 ± 2.68) was significantly higher than those of AL (1.96 ± 0.35) and others (2.00 ± 0.74) (all p < 0.05). SUVmax of grade 3 (8.95 ± 1.89) and grade 2 (4.71 ± 0.23) were also significantly higher than those of grade 1 (1.92 ± 0.31) and grade 0 (1.59 ± 0.39) (all p < 0.05). Correlation coefficient (R2) of SUVmax reached 0.966 to 0.978 with only small systematic difference (intra = − 0.14; inter = − 0.23) between two repeated measurements. Intra- and inter-operator RPCs were 0.688 and 0.877. Conclusions 99mTc-PYP quantitative SPECT integrated with adjustable PVC factors is feasible to quantitatively and objectively assess the burden of cardiac amyloidosis for diagnosis of ATTR-CM.


2021 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Temidayo Abe ◽  
Titilope Olanipekun ◽  
Mtanis Khoury ◽  
Obiora Egbuche ◽  
Effoe Valery ◽  
...  

2018 ◽  
Vol 93 (10) ◽  
pp. 1207-1210 ◽  
Author(s):  
Marcella Tschautscher ◽  
Vincent Rajkumar ◽  
Angela Dispenzieri ◽  
Martha Lacy ◽  
Morie Gertz ◽  
...  

2018 ◽  
Vol 20 (4) ◽  
pp. 486-486
Author(s):  
Olena Nemchyna ◽  
Rolf Gebker ◽  
Felix Schoenrath ◽  
Christoph Knosalla ◽  
Volkmar Falk ◽  
...  

2018 ◽  
Vol 49 (1) ◽  
pp. 9-14
Author(s):  
Monika Adamska ◽  
Anna Komosa ◽  
Tatiana Mularek ◽  
Joanna Rupa-Matysek ◽  
Lidia Gil

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.


Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 5519-5519
Author(s):  
Jinuo Wang ◽  
Jian-Hua Han ◽  
Yue-lun Zhang ◽  
Xin-xin Cao ◽  
Dao-Bin Zhou ◽  
...  

Introduction Monoclonal gammopathy of undetermined significance (MGUS) is a clinically asymptomatic premalignant plasma cell disorder. Previous studies in Western countries have described the prevalence of MGUS in Caucasians. However, data is limited in Chinese population. We therefore performed this study to ascertain the prevalence and characteristics of MGUS among Chinese population. Methods A total of 154597 consecutive healthy participants from Beijing who underwent annual physical examination between December 2013 and April 2019 at Peking Union Medical College Hospital were enrolled. Serum M protein was evaluated by capillary electrophoresis. Patients with a positive or suspicious serum M protein were suggested to be referred to the hematological clinic for immunofixation electrophoresis (IFE) and free light chain (FLC) assays. MGUS was defined in accordance with previous definitions. We calculated age-specific and sex-specific prevalence and described laboratory characteristics of patients with MGUS among those participants. Results MGUS were diagnosed in 843 patients (0.55%, 95%CI 0.51% to 0.59%). The median age at presentation was 58 years, with a range of 25-96 years. The overall prevalence of MGUS was 1.14% among participants aged 50 years or older and 2.6% among those aged 70 years or older. In both sexes, the prevalence increased with age: 0.1% (<40 years), 0.36% (40-49 years), 0.78% (50-59 years), 1.28% (60-69 years), 2.19% (70-79 years), and 3.77% (≥80 years) separately (Figure 1). The prevalence among men were higher than that among women (0.67% vs. 0.40%, OR =1.719, 95% CI 1.490 to 1.983, P<0.001) (Figure 1). The median concentration of serum Monoclonal protein was 1.4 g/L (0.1 -27.8 g/L). M protein level was less than 0.5g/L in 220 patients (26.1%), less than 5 g/L in 81.1% and more than 15 g/L in only 1.9% of 843 persons. There was no significant difference in the concentration of the monoclonal protein among the age groups. Of the 519 patients who were tested for IFE, the isotype of the monoclonal immunoglobulin was IgG in 344 (66.3%), IgA 112 (21.6%), IgM in 48 (9.2%), IgD in 2 (0.4%), light-chain in 3 (0.6%) and biclonal in 10 (1.9%). The serum light-chain type was kappa in 260 (50.1%), lambda in 255 (49.1%) patients, while 4 patients (0.8%) with biclonal M protein have both kappa and lambda light-chain. Of the 180 people who were tested for FLC, 42 (23.3%) had an abnormal FLC ratio. IgG isotype, M protein <15 g/L and normal FLC ratio were found in 102 patients (56.7%) and the remaining 78 people (43.4%) had 1(30.6%) or 2(12.8%) abnormal factors. Conclusions MGUS was found in 1.14% of persons 50 years of age or older and 2.6% among those 70 years of age or older among healthy Chinese population. The prevalence of MGUS increases with age. Males have a higher frequency of MGUS than Females. These observations offer the overall situation of MGUS epidemiology in a large Chinese population. Disclosures No relevant conflicts of interest to declare.


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