Light chain amyloidosis: Experience in a tertiary hospital: 2005-2013

I Krsnik; M Cabero; D Morillo; J Segovia; P García-Pavía; M Gómez-Bueno; C Salas

doi:10.1016/j.rce.2014.08.013

Light chain amyloidosis: Experience in a tertiary hospital: 2005-2013

Rev Clin Esp (Barc). 2015 Jan-Feb;215(1):1-8. doi: 10.1016/j.rce.2014.08.013. Epub 2014 Oct 11.

[Article in English, Spanish]

Authors

I Krsnik¹, M Cabero², D Morillo², J Segovia³, P García-Pavía³, M Gómez-Bueno³, C Salas⁴

Affiliations

¹ Servicio de Hematología, Hospital Universitario Puerta de Hierro-Majadahonda, Majadahonda, Madrid, España. Electronic address: isabel.krsnik@salud.madrid.org.
² Servicio de Hematología, Hospital Universitario Puerta de Hierro-Majadahonda, Majadahonda, Madrid, España.
³ Servicio de Cardiología, Hospital Universitario Puerta de Hierro-Majadahonda, Majadahonda, Madrid, España.
⁴ Servicio de Anatomía Patológica, Hospital Universitario Puerta de Hierro-Majadahonda, Majadahonda, Madrid, España.

PMID: 25308790
DOI: 10.1016/j.rce.2014.08.013

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.

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.