Abstract：
BACKGROUND AND OBJECTIVES:AL amyloidosis is a rare condition whose management is undergoing changes due to recent advances in diagnosis and treatment. We describe a contemporary series of patients with AL amyloidosis to analyze the features that enable early diagnosis and optimal management. PATIENTS AND METHODS:We recruited for analysis 32 patients (19 women; mean age, 63 years) treated consecutively at our center. RESULTS:Eighty-four percent of the patients presented with asthenia, dyspnea or edema, with a previous duration of symptoms of 8 months (median). Cardiac (21/32) and renal impairment were the most common type (11/32). All of the patients, except one, had a monoclonal component in serum/urine or abnormal values for free light chains (78%, λ). The bone marrow (BM) showed clonal plasmacytosis in 29 cases. All of the cardiac biopsies and 50% of the BM biopsies showed amyloid deposits. The results of the echocardiogram and/or cardiac resonance were abnormal in 27/30 cases. The median NT-proBNP value at diagnosis was 5200 ng/ml. Thirteen patients died due to heart failure, 2 due to rejection after heart transplantation, 2 due to pneumonia and 1 after a stroke. Ten patients did not undergo treatment, 12 were treated with bortezomib and 5 were treated with alkylating agents. Five patients underwent heart transplantation and 4 underwent autologous bone marrow transplantation. Fourteen patients achieved a complete hematologic response and 10 achieved organ response. The median survival was 17 months. CONCLUSIONS:Cardiac involvement is the major determinant of prognosis. Yield of involved organ biopsy is high (100% heart biopsies). Antineoplastic treatment with bortezomib and/or autologous bone marrow transplantation achieves hematological responses with improvements in organ impairment.
journal_name
Rev Clin Espjournal_title
Revista clinica espanolaauthors
Krsnik I,Cabero M,Morillo D,Segovia J,García-Pavía P,Gómez-Bueno M,Salas Cdoi
10.1016/j.rce.2014.08.013subject
Has Abstractpub_date
2015-01-01 00:00:00pages
1-8issue
1eissn
0014-2565issn
1578-1860pii
S0014-2565(14)00349-Xjournal_volume
215pub_type
杂志文章abstract：BACKGROUND:Sarcopenia is characterized by the loss of skeletal muscle mass and strength and represents a risk factor for frailty, loss of independence and physical disabilities. This study aimed to assess the prevalence of sarcopenia in a population of patients admitted to a media and long-stay unit. PATIENTS AND METH...
journal_title：Revista clinica espanola
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journal_title：Revista clinica espanola
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journal_title：Revista clinica espanola
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journal_title：Revista clinica espanola
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doi：
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abstract：:Central venous catheters are essential in modern-day medical practice, particularly in intensive care units. Although such catheters provide necessary vascular access, their use puts patients at risk of mechanical and infectious complications. Unfortunately, these complications are associated with an increase of the m...
journal_title：Revista clinica espanola
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journal_title：Revista clinica espanola
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journal_title：Revista clinica espanola
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journal_title：Revista clinica espanola
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journal_title：Revista clinica espanola
pub_type： 杂志文章
doi：
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journal_title：Revista clinica espanola
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journal_title：Revista clinica espanola
pub_type： 杂志文章
doi：
更新日期：1991-01-01 00:00:00
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journal_title：Revista clinica espanola
pub_type： 杂志文章
doi：
更新日期：1995-03-01 00:00:00
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journal_title：Revista clinica espanola
pub_type： 杂志文章
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journal_title：Revista clinica espanola
pub_type： 杂志文章
doi：
更新日期：1993-09-01 00:00:00
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journal_title：Revista clinica espanola
pub_type： 杂志文章,评审
doi：
更新日期：1992-05-01 00:00:00
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journal_title：Revista clinica espanola
pub_type： 杂志文章
doi：
更新日期：1991-03-01 00:00:00
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journal_title：Revista clinica espanola
pub_type： 杂志文章
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journal_title：Revista clinica espanola
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doi：
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journal_title：Revista clinica espanola
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doi：
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journal_title：Revista clinica espanola
pub_type： 杂志文章,评审
doi：
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journal_title：Revista clinica espanola
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更新日期：2002-04-01 00:00:00
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journal_title：Revista clinica espanola
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doi：10.1157/13062267
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journal_title：Revista clinica espanola
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doi：
更新日期：1996-04-01 00:00:00
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journal_title：Revista clinica espanola
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doi：10.1016/s0014-2565(02)71048-5
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journal_title：Revista clinica espanola
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doi：
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journal_title：Revista clinica espanola
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doi：
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