scholarly journals A Case Report of Cardiac Amyloidosis Highlighting the Importance of Strain Analysis

2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Andres Cordova Sanchez ◽  
Ryan Murphy ◽  
Suman Rao ◽  
Fidel Martinez ◽  
Stephanie Bryant ◽  
...  
Keyword(s):  
Heart Failure ◽  
Case Report ◽  
High Mortality ◽  
Light Chain ◽  
Cardiac Amyloidosis ◽  
Inverse Relationship ◽  
Cardiac Involvement ◽  
Strain Analysis ◽  
Al Amyloidosis ◽  
Cardiac Symptoms

Cardiac involvement in light-chain (AL) amyloidosis has a high mortality. Once cardiac symptoms are present, it is important to make a diagnosis as there is an inverse relationship between mortality and time of diagnosis. Echocardiography is usually one of the first tests performed. But strain analysis, which can provide important clues, is not routinely performed. This is a case of AL amyloidosis presenting with heart failure in which echocardiographic strain analysis was vital for its diagnosis.

scholarly journals Efficacy and Tolerability of Daratumumab in Heavily Pretreated AL Amyloidosis: A Systematic Review

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 2025-2025 ◽  
Author(s):  
Muhammad Aadil Rahman ◽  
Ali Younas Khan ◽  
Awais Ijaz ◽  
Muhammad Junaid Tariq ◽  
Muhammad Usman ◽  
...  
Keyword(s):  
Heart Failure ◽  
Partial Response ◽  
Light Chain ◽  
Cardiac Amyloidosis ◽  
Cardiac Involvement ◽  
Renal Involvement ◽  
Renal Amyloidosis ◽  
Al Amyloidosis ◽  
Heavily Pretreated ◽  
Organ Response

Abstract Introduction Light chain (AL) amyloidosis is a low burden plasma cell disorder, characterized by deposition of misfolded lambda or kappa light chains. Kidney dysfunction is present in almost two-thirds of patients at the time of initial presentation, followed by diastolic heart failure in about 50% of cases, which is responsible for 75% of deaths in these patients. Autologous stem cell transplant (auto-SCT) remains the gold standard for the management of AL amyloidosis but is often impractical to perform by virtue of patients' age, medical comorbidities including cardiac involvement. Methods We conducted a literature search using three databases (PubMed, Embase,Web of Science). Our search strategy included MeSH terms and key words such as AL amyloidosis, daratumumab and darzalex from date of inception to March 2018. After excluding duplicates, reviews and non-relevant articles, we selected eight studies, including two case reports, two phase II prospective trials and four retrospective trials. Results Data on 129 patients was included, there ages ranged from 43-83 years. Median number of prior therapies were 3 (range: 2-6), 106 (82%) received proteasome inhibitor (bortezomib) based therapy, and 69 (53.5%) received immunomodulatory (lenalidomide) based therapy. Another 41 (32%) received high dose melphalan (HDM) followed by auto-SCT. The time from the diagnosis of AL to the start of daratumumab therapy varied from 0.7-150 months. Eighty-nine (69%) patients had cardiac and 64 (49.6%) patients had renal involvement. A total of 114 (88%) patients received a daratumumab dose of 16 mg/kg weekly for 8 weeks followed by every 2 weeks for the next 8 weeks. A total of 104 patients were evaluable for hematological response, assessed by improvement in free light chain (FLC) levels. Daratumamab achieved an impressive overall response rate (ORR) of 72% (n=75). Complete remission (CR) in 15 (14%) of patients, very good partial response (VGPR) in 44 (42%) and a partial response (PR) in 16 (15%) of patients was noted. Thirty-four patients with cardiac involvement and 26 patients with renal amyloidosis were assessed for organ response across four studies. Thirteen (38%) patients with cardiac amyloidosis demonstrated an improvement in N-terminal pro brain natriuretic peptide (NT-proBNP) levels. Ten (38%) patients with renal involvement responded according to consensus criteria [Palladini et al 2014] for organ response. Another two had improvement in serum creatinine levels. Among the 129 patients treated with daratumumab for AL amyloidosis, 36 (32%) reported infusion related reactions (IRR). Most were mild (grade 1-2). Daratumumab infusion was well tolerated in patients with cardiac (n=54) and renal involvement (n=48). Only one patient needed adjustment in his diuretic dose, another one developed decompensated heart failure and one died due to progression of cardiac disease. Seven patients had worsening of their NT-proBNP levels. Similarly, no dose adjustments were required for patients with renal amyloidosis and one patient tolerated daratumumab infusion at a GFR<20 mL/min without any complications. Conclusion Daratumumab monotherapy is associated with deep and prompt hematological responses in patients with heavily pretreated AL amyloidosis, at the standard dosing regimens used for multiple myeloma, with a favorable safety profile. Furthermore, daratumumab performed well in patients with cardiac amyloidosis even though there is an increased risk of volume overload and infusion related morbidity. Given the high incidence of peripheral neuropathy with bortezomib, cardiotoxicity with carfilzomib based regimens in amyloidosis patients, daratumumab appears to be a suitable alternative. It has already been approved for relapsed amyloidosis (AL) patients in the European Union. Currently, it is being investigated as monotherapy for AL amyloidosis in phase 2 trials (NCT02841033 and NCT02816476) and in combination with bortezomib, cytoxin and dexamethasone (VCd) in a phase III trial (NCT03201965). Disclosures No relevant conflicts of interest to declare.

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.

scholarly journals Comparison of Different Techniques to Identify Cardiac Involvement in Immunoglobulin Light Chain Amyloidosis

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 3182-3182
Author(s):  
Mohammed A Aljama ◽  
M Hasib Sidiqi ◽  
Angela Dispenzieri ◽  
Morie A. Gertz ◽  
Martha Q. Lacy ◽  
...  
Keyword(s):  
Strain Rate ◽  
Board Of Directors ◽  
Light Chain ◽  
Mayo Clinic ◽  
Endomyocardial Biopsy ◽  
Research Funding ◽  
Cardiac Amyloidosis ◽  
Cardiac Involvement ◽  
Al Amyloidosis ◽  
Advisory Committees

Abstract Background: Cardiac involvement is integral in staging and prognosis of immunoglobulin light chain (AL) amyloidosis. The N-terminal prohormone of brain natriuretic peptide (NT proBNP) is a cardiac biomarker used in screening for cardiac involvement and staging the disease. Transthoracic echocardiogram (TTE) and cardiac magnetic resonance (CMR) are the imaging modalities recommended to determine cardiac involvement and function. Methods: We conducted a retrospective review of all patients with biopsy proven systemic AL amyloidosis seen at the mayo clinic between Jan 1, 2006 and Dec 30, 2015. The aim of the study is to identify the nature of abnormalities in cardiac biomarkers and echocardiographic features in patients with AL amyloidosis and the ability of these investigations to diagnose cardiac involvement. We first identified all patients with AL amyloidosis that underwent endomyocardial biopsy for suspicion of cardiac involvement (Cohort 1). We then analyzed a cohort (Cohort 2) which consisted of patients who had serum NT proBNP and a comprehensive echocardiographic evaluation at time of diagnosis. Results: 179 patients with AL amyloidosis underwent endomyocardial biopsy (Cohort 1) of whom 173 had evidence of amyloid deposition. In this cohort, 159 patients had NT proBNP performed at the time of the procedure. The NT proBNP was elevated (>300) in all 159 patients with a median NT proBNP of 4917 (range 355-69541). The median left ventricular ejection fraction (LVEF), interventricular septal (IVS) thickness and strain rate were 54 (range 10-77), 15 (range 8-30) and -9 (range -21 to 0) respectively. CMR findings were consistent or suggestive of light chain amyloidosis in 38/42 patients, yielding a sensitivity of 90 percent. The LVEF, IVS thickness and strain rate were abnormal in 89/168 (53%), 102/64 (61%) and 92/95 (97%) respectively. 95 patients with biopsy proven cardiac amyloidosis had complete echocardiogram data available on LVEF, IVS thickness and strain rate, with 97% (n=92) presenting with an abnormality in at least one of these variables . CMR findings were consistent or suggestive of light chain amyloidosis in 38/42 patients, yielding a sensitivity of 90 percent. Patients with a normal NT proBNP and normal echocardiogram were considered disease free (true negative), based on our initial analysis of these investigations in Cohort 1. Cohort 2 consisted of 342 consecutive patients. The median NT pro BNP was 1878 (25-48214). The median LVEF, IVS thickness and strain rate were 63 (22-90), 14 (6-25) and -13 (-25 to -3) respectively. 259 (76%) patients had a positive NT proBNP (above 300), of whom 237 (92%) had an abnormality detected on TTE. 83 patients had a negative NT proBNP, of whom 27 (33%) had an abnormality in either LVEF, IVS thickness or strain rate. 19 of these 27 patients had a borderline reduced strain rate between -17 and -18, whilst the remaining 8 patients had a strain between -14 and -15. Only 6/27 patients were considered to have possible early cardiac involvement and none have any other diagnostic or classical features of amyloidosis on TTE. Conclusion: The combination of NT proBNP and comprehensive echocardiographic evaluation provides substantial information to diagnose cardiac amyloidosis in a significant portion of patients negating the need for endomyocardial biopsy. A negative NT proBNP rules out clinically meaningful cardiac involvement and may obviate the routine use of TTE in patients with a low clinical suspicion of cardiac amyloidosis. Disclosures Dispenzieri: Celgene, Takeda, Prothena, Jannsen, Pfizer, Alnylam, GSK: Research Funding. Gertz:Research to Practice: Consultancy; Physicians Education Resource: Consultancy; Ionis: Honoraria; celgene: Consultancy; spectrum: Consultancy, Honoraria; Teva: Consultancy; Amgen: Consultancy; Medscape: Consultancy; janssen: Consultancy; Alnylam: Honoraria; Abbvie: Consultancy; annexon: Consultancy; Apellis: Consultancy; Prothena: Honoraria. Lacy:Celgene: Research Funding. Dingli:Millennium Takeda: Research Funding; Millennium Takeda: Research Funding; Alexion Pharmaceuticals, Inc.: Other: Participates in the International PNH Registry (for Mayo Clinic, Rochester) for Alexion Pharmaceuticals, Inc.; Alexion Pharmaceuticals, Inc.: Other: Participates in the International PNH Registry (for Mayo Clinic, Rochester) for Alexion Pharmaceuticals, Inc.. Kapoor:Takeda: Research Funding; Celgene: Research Funding. Kumar:AbbVie: Membership on an entity's Board of Directors or advisory committees, Research Funding; Novartis: Research Funding; Roche: Research Funding; Celgene: Membership on an entity's Board of Directors or advisory committees, Research Funding; Oncopeptides: Membership on an entity's Board of Directors or advisory committees; Celgene: Membership on an entity's Board of Directors or advisory committees, Research Funding; KITE: Membership on an entity's Board of Directors or advisory committees, Research Funding; Takeda: Membership on an entity's Board of Directors or advisory committees; Janssen: Membership on an entity's Board of Directors or advisory committees, Research Funding; Janssen: Membership on an entity's Board of Directors or advisory committees, Research Funding; AbbVie: Membership on an entity's Board of Directors or advisory committees, Research Funding; KITE: Membership on an entity's Board of Directors or advisory committees, Research Funding; Merck: Membership on an entity's Board of Directors or advisory committees, Research Funding.

scholarly journals Insight from an autopsy in a patient with rapidly worsening heart failure due to amyloid light-chain cardiac amyloidosis: A case report

2020 ◽  
Vol 22 (2) ◽  
pp. 48-51
Author(s):  
Hiroaki Yokoyama ◽  
Koki Shishido ◽  
Junko Ito ◽  
Wataru Kamata ◽  
Nagaaki Katoh ◽  
...  
Keyword(s):  
Heart Failure ◽  
Case Report ◽  
Light Chain ◽  
Cardiac Amyloidosis ◽  
Amyloid Light Chain

Efficacy and Toxicity of Dose-Reduced Bortezomib/Dexamethasone Chemotherapy In Patients with High Risk Cardiac Light Chain Amyloidosis (Mayo Clinic stage III)

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 1960-1960 ◽  
Author(s):  
Stefan O Schonland ◽  
Tilmann Bochtler ◽  
Anthony D. Ho ◽  
Ute Hegenbart
Keyword(s):  
Light Chain ◽  
Mayo Clinic ◽  
Cardiac Amyloidosis ◽  
Cardiac Involvement ◽  
Early Death ◽  
Stage Iii ◽  
Al Amyloidosis ◽  
Hematologic Toxicity ◽  
Newly Diagnosed ◽  
Grade 3

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.

scholarly journals Another Obesity Paradox: In Cardiac AL Amyloid a Higher BMI Is Associated with a Lower Rate of Cardiac Response

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 5502-5502
Author(s):  
So Yeon Kim ◽  
Amanda Vest ◽  
Raymond L Comenzo ◽  
Cindy Varga
Keyword(s):  
Heart Failure ◽  
Board Of Directors ◽  
Light Chain ◽  
Research Funding ◽  
Cardiac Amyloidosis ◽  
Cardiac Response ◽  
Al Amyloidosis ◽  
Advisory Committees ◽  
Hematological Response ◽  
High Bmi

Background:Light-chain amyloidosis (AL) is a clonal plasma cell disorder in which Ig light chains cause organ-specific disease due to toxic misfolded light-chain aggregates and extracellular deposition of amyloid fibrils derived from light chain proteins. The majority of amyloid patients present in various stages of heart failure and survival is largely driven by the extent of cardiac involvement. In the general heart failure population, overweight and mild/moderate obesity is associated with lower mortality, termed the obesity survival paradox. Conversely for patients with multiple myeloma, a disease similar in pathophysiology to AL, obesity is a risk factor for hematological progression. Hypothesis:We hypothesized that patients with cardiac amyloidosis would exhibit an obesity survival paradox and sought to determine the impact of BMI on hematological and cardiac responses to anti-plasma cell treatment. Methods:We conducted a single tertiary center retrospective study of consecutive patients with cardiac AL amyloidosis, referred between 1/1/2009 and 09/30/2018. We collected demographics and BMI prior to treatment. We recorded the date of diagnosis and subsequent dates of hematological and/or cardiac response, mortality or end of follow-up. We constructed a Cox proportional hazards model examining the association between BMI and mortality with a restricted cubic spline function curve. Three logistic regression models were constructed to examine the association between high BMI (>/=25 kg/m2) and cardiac or hematological response, and mortality. Models were adjusted for age, sex and cardiac stage at the time of diagnosis. Results:Of 79 patients, 17 patients had BMI of 17-22.5, 19 a BMI of 22.6-25, 23 a BMI of 25.1-29.7, and 20 a BMI of >/=30 kg/m2. Crude mortality was 31/79 (39%). There was no relationship between BMI as a continuous variable and mortality (HR 0.98, 95% CI 0.91-1.06, p=0.625, adjusted for age and sex), although a survival paradox trend was suggested by the spline curve. While there was no relationship between high BMI and hematological response (adjusted OR 1.00, 0.37-2.75, p=0.996), there was a relationship between high BMI and lower likelihood of achieving cardiac response (adjusted OR 0.23, 0.07-0.71, p=0.011). Conclusions:In this small cohort of patients with AL cardiac amyloidosis, there was no significant relationship between BMI and mortality. Hematological response was unrelated to BMI, but patients with a higher BMI were significantly less likely to achieve a cardiac response. These findings suggest that obesity might be associated with poorer cardiac outcomes in AL amyloidosis, highlighting the importance of a multidisciplinary approach involving oncologists, cardiologists, and nutritionists in the treatment of this complex multi-organ disease. Disclosures Comenzo: Caelum: Consultancy, Membership on an entity's Board of Directors or advisory committees; Prothena Biosciences: Consultancy, Membership on an entity's Board of Directors or advisory committees, Research Funding; Sanofi-Aventis: Membership on an entity's Board of Directors or advisory committees; Takeda: Research Funding; Unum: Membership on an entity's Board of Directors or advisory committees, Research Funding; Myself: Patents & Royalties: Patent 9593332, Pending 20170008966; Janssen: Consultancy, Membership on an entity's Board of Directors or advisory committees, Research Funding; Karyopharm: Research Funding.

scholarly journals P1684 Cardiac amyloidosis hits the valve first

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
S Iakovlev ◽  
A I Kalinskaya
Keyword(s):  
Heart Failure ◽  
Case Report ◽  
Tricuspid Valve ◽  
Cardiac Amyloidosis ◽  
Global Longitudinal Strain ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Al Amyloidosis ◽  
Ventricular Ejection Fraction ◽  
Arterial Blood

Abstract Case report. 60-year-old man without previous history of coronary artery disease was admitted to our hospital. He complained of weakness, low extremities edema. Physical examination revealed also enlargement of the liver, positive hepatojugular reflux, multiple ecchymoses on patient’s face, especially in periorbital area. The arterial blood pressure was 100/60 mm Hg. ECG showed sinus rhythm 83/min, low R waves in V2, V3 with biphasic T waves in V4-V6. The last coronary angiography revealed normal coronary arteries, it was performed 6 months ago because of atypical chest pain and inconclusive stress test. Echocardiography revealed severe tricuspid regurgitation (TR) due to leaflets restriction and malcoaptation. TR gradient was about 10 mm Hg. Interventricular septum 12 mm, left ventricle posterior wall 9 mm, mass index 87 g/m2, relative wall thickness 0.38; no LV regional wall motion abnormalities was noted, left ventricular ejection fraction (LV EF) was about 50%, mitral valve E/A ratio was 1,4, but average E/E" = 17. Left atrium volume 33 ml/m2. Insignificant amount of pericardial effusion also was found. 3D evaluation of tricuspid valve (TV) showed no leaflets defects and chordal ruptures. LV global longitudinal strain (LV GLS) was – 11,1 % with the apex/(mid + base) ratio 1,3 - apical sparing pattern. Cardiac MRI with gadolinium also showed severe TR and diffuse late subendocardial gadolinium enhancement in both ventricles. We suspected cardiac amyloidosis with significant tricuspid valve involvement, torrential TR, and right ventricle volume overload. The rectum biopsy was negative. The cardiac muscle biopsy with congo red straining was positive for amyloidosis. The patient was transferred to hematology clinic where the diagnosis of AL-amyloidosis was confirmed. The treatment with lenalidomide and prednisone was started. Unfortunately, one month later the patient died. The autopsy was not performed due to religious reasons. Discussion AL-amyloidosis is a systemic disease characterized by multiple organ and tissue changes and associated with poor prognosis. Cardiac involvement is a major prognostic factor as it accounts for approximately 75% of death due to heart failure or arrhythmias. Infiltration of myocardium with amyloid leads to diastolic than systolic dysfunction of the heart and to developing of the heart failure. The most common presentation of heart involvement in AL-amyloidosis is fatigue and dyspnea. In this case the main complaint of the patient was peripheral edema, echocardiography showed damaged TV and preserved LV systolic function. In literature, we found only one case report describing cardiac amyloidosis presented as severe TR. Conclusion In patients presenting with significant isolated valvular dysfunction and heart failure the cardiac amyloidosis can be suspected. The comprehensive echocardiography is the most useful tool to detect this problem. Abstract P1684 Figure. 3D picture of tricuspid valve

P29 Arrest of progression of cardiac amyloidosis after chemotherapy predicts favorable outcome in patients with light-chain amyloidosis

2020 ◽  
Vol 41 (Supplement_1) ◽  
Author(s):  
J Koyama ◽  
M Minamisawa ◽  
K Kuwahara
Keyword(s):  
Wall Thickness ◽  
Light Chain ◽  
Cardiac Amyloidosis ◽  
Cardiac Involvement ◽  
Left Ventricular ◽  
Favorable Outcome ◽  
Rank Test ◽  
Al Amyloidosis ◽  
Interval Change ◽  
Light Chain Amyloidosis

Abstract Funding Acknowledgements none Background Many studies demonstrated that cardiac involvement predicts poor prognosis in patients with systemic light-chain amyloidosis (AL). There is no data about the effect of the arrest of progression of cardiac amyloidosis on prognosis after chemotherapy. Hypothesis Arrest of progression of cardiac amyloidosis is associated with favorable outcome in patients with light-chain amyloidosis. Methods Among 126 consecutive patients with AL, we prospectively examined 94 patients serially after optimal therapy. The mean follow-up period was 1405 ± 1510 days (median value 734 days, inter quartile range 176-2343 days). Wall thickness was measured from left ventricular (LV) m-mode trace. We defined the cardiac involvement as mean LV wall thickness &gt;12mm, and the regression or progression of cardiac amyloidosis as change in LV mean wall thickness &gt;1mm. Results Among 94 patients with AL, 28 patients (30%) showed regression by definition above, 35 patients (37%) showed no interval change and 31 patients (33%) showed progression of cardiac amyloidosis. Survival analysis of 3 groups demonstrated that the regression and arrest of progression groups showed better outcome compared with the progression group (Log-rank test P &lt; 0.0001). Conclusions The arrest of progression of cardiac amyloidosis predicts favorable outcome in patients with AL amyloidosis. Abstract P29 Figure. Kaplan-Meier Curve of 3 groups

Treatment with doxorubicin-based protocol in cardiac involvement diffuse large B-cell lymphoma: A case report

2021 ◽  
pp. 107815522110351
Author(s):  
Atakan Tekinalp ◽  
Taha U Kars ◽  
Hatice Z Dikici ◽  
Pınar D Yılmaz ◽  
Sinan Demircioğlu ◽  
...  
Keyword(s):  
Heart Failure ◽  
Case Report ◽  
B Cell ◽  
Cardiac Involvement ◽  
Cell Lymphoma ◽  
Vena Cava ◽  
Standardized Uptake Value ◽  
B Cell Lymphoma ◽  
Large B Cell Lymphoma ◽  
Large B Cell

Introduction Cardiac involvement in diffuse large B-cell lymphoma is a rare entity in non-Hodgkin lymphomas. Symptoms are usually related to heart failure. Patients who are severely symptomatic due to cardiac mass could be considered treatment as soon as possible. In this report, we present a patient diagnosed with diffuse large B-cell lymphoma with cardiac involvement. Case Report A 61-year-old female patient was admitted to our unit with gastric biopsy diffuse large B-cell lymphoma. Computerized tomography of the chest and positron emission tomography/computed tomography demonstrated a neoplastic mass in the intra-atrial septum extended to inferior vena cava (5 × 4 cm in size and standardized uptake value maximum 24.6). She was in stage III and in the high-risk group. Because of pronounced heart failure findings associated with the mass-specific chemotherapy was planned early. Management & Outcome Although a fraction of ejection was 60% by echocardiography before the treatment, she had a cardiac risk for doxorubicin due to being over 60 years old and hypertension. Complete remission was achieved after three cycles of rituximab–cyclophosphamide–doxorubicin–vincristine and methylprednisolone protocol including doxorubicin. Treatment was completed with six cycles and she was followed up for three months. Discussion Because of the cardiotoxicity of doxorubicin-based protocols, patients should be evaluated according to cardiac functions before and during the chemotherapy.

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