[The clinical features and outcomes of immunoglobulin light-chain amyloidosis with heart involvement].
ABSTRACT To analyze the clinical features and outcomes of patients with immunoglobulin light-chain amyloidosis (AL) who had heart involvement.
Clinical features and outcomes of AL amyloidosis patients with heart involvement in the past 7 years in our hospital were retrospectively analyzed.
Cardiac involvement was seen in 36 out of the 60 AL patients (60%). The clinical manifestations of cardiac amyloidosis included heart failure (50%), low QRS voltage (47.2%) and pseudomyocardial infarction (33.3%) in electrocardiography, as well as thickening of ventricular wall (63.9%), echo of granular sparkling texture (11.1%), atria dilation (33.3%) and diastolic dysfunction (30.6%) in echocardiography. The prognosis was poor, with a median survival time of 13.9 months.
Patients of AL amyloidosis with cardiac involvement are not rare. Thickening of ventricular wall and diastolic dysfunction are the most common characteristics. Special attention should be paid to this disease.
- SourceAvailable from: Emanuele Cereda[Show abstract] [Hide abstract]
ABSTRACT: Nutritional status is an independent prognostic factor in immunoglobulin light-chain amyloidosis (AL), but its influence on quality of life (QoL) is unknown. The aim of this cross-sectional study was to investigate the association between nutritional status and QoL in AL patients at diagnosis. One hundred and fifty consecutive patients with biopsy-proven AL were assessed for nutritional status by anthropometry [body mass index, unintentional weight loss (WL) in the previous 6 months and mid-arm muscle circumference (MAMC)], biochemistry (serum prealbumin), and semiquantitative food intake at referral. QoL was assessed by the Medical Outcomes Study 36-item Short Form General Health Survey. The composite physical component summary (PCS) and the mental component summary (MCS) for AL outpatients were 36.2 ± 10.1 and 44.9 ± 11.3, respectively (p < 0.001 for both vs the population norms of 50). In multivariate linear regression models adjusted for gender, age, Eastern Cooperative Oncology Group performance status, the number of organs involved, the severity of cardiac damage, C-reactive protein, energy intake, and WL, PCS was significantly lower for serum prealbumin <200 mg/L and MAMC <10th percentile (adjusted difference 3.8, 95% CI 0.18-7.5, p = 0.040 and 5.3, 95% CI 2.0-8.7, p = 0.002, respectively). MCS was decreased by 0.47 (95% CI 0.18-0.75, p = 0.002) for each kilogram of body weight lost in the previous 6 months. Nutritional status independently affects QoL in AL patients since diagnosis. Nutritional evaluation should be integral part of the clinical assessment of AL patients. Nutritional support intervention trials are warranted in such patients' population.Annals of Hematology 08/2011; 91(3):399-406. · 2.87 Impact Factor
- [Show abstract] [Hide abstract]
ABSTRACT: We report a case of a 51-year-old Caucasian man with progressive heart failure and multiple lytic lesions in the skull and long bones. The diagnosis of multiple myeloma and cardiac involvement by secondary amyloidosis was made, but the patient died despite treatment, and the diagnosis was confirmed at autopsy. Cardiac amyloidosis is clinically silent and when the symptoms appear, median survival is less than 1 year. The diagnosis of amyloidosis should be considered in any patient with multiple myeloma and heart failure, and the magnetic resonance imaging (MRI) can help to make this diagnosis.Boletín de la Asociación Médica de Puerto Rico 01/2013;
Article: Effusive reasoning.[Show abstract] [Hide abstract]
ABSTRACT: KEY WORDSpattern recognition–analytic reasoning–problem representation–clinical diagnostic reasoningJournal of General Internal Medicine 07/2011; 26(10):1204-8. · 3.28 Impact Factor