title: Light chain amyloidosis: Experience in a tertiary hospital: 2005-2013.
abstract: OBJECTIVE
  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.
  
  
  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.
authors.affiliation: Servicio de Hematología, Hospital Universitario Puerta de Hierro-Majadahonda, Majadahonda, Madrid, España. Electronic address: isabel.krsnik@salud.madrid.org.
authors.forename: I
authors.initials: I
authors.lastname: Krsnik
authors.affiliation: Servicio de Hematología, Hospital Universitario Puerta de Hierro-Majadahonda, Majadahonda, Madrid, España.
authors.forename: M
authors.initials: M
authors.lastname: Cabero
authors.affiliation: Servicio de Hematología, Hospital Universitario Puerta de Hierro-Majadahonda, Majadahonda, Madrid, España.
authors.forename: D
authors.initials: D
authors.lastname: Morillo
authors.affiliation: Servicio de Cardiología, Hospital Universitario Puerta de Hierro-Majadahonda, Majadahonda, Madrid, España.
authors.forename: J
authors.initials: J
authors.lastname: Segovia
authors.affiliation: Servicio de Cardiología, Hospital Universitario Puerta de Hierro-Majadahonda, Majadahonda, Madrid, España.
authors.forename: P
authors.initials: P
authors.lastname: García-Pavía
authors.affiliation: Servicio de Cardiología, Hospital Universitario Puerta de Hierro-Majadahonda, Majadahonda, Madrid, España.
authors.forename: M
authors.initials: M
authors.lastname: Gómez-Bueno
authors.affiliation: Servicio de Anatomía Patológica, Hospital Universitario Puerta de Hierro-Majadahonda, Majadahonda, Madrid, España.
authors.forename: C
authors.initials: C
authors.lastname: Salas
keywords: Amiloidosis; Amyloidosis; Autologous progenitor transplantation; Bortezomib; Cadenas ligeras libres; Fragmento aminoterminal del péptido natriurético cerebral; Free light chains; Heart transplantation; N-terminal prohormone of brain natriuretic peptide; Trasplante autólogo de progenitores; Trasplante cardiaco