RESUMEN
The basic principles of lymphoma classification(s) in general have been widely evolving in aâ¯course of decades of years wiht the use of contemporary resources and recent cutting edges in hematooncology on aâ¯clinical, morphological and molecular level bring new possibilities not only in improvements of diagnostic and prognostic algorithms and also bear new opportunities in so called targeted and tailored strategies of lymphoma therapy. The pathogenesis and biologic behavior of lymphoproliferations and even lymphomas should be studied in aâ¯context of lymphocytic and (neoplastic) lymphoid stage and chronologic development. In aâ¯current more complex insight into lymphoproliferations we would like to describe huge heterogeneity of diffuse large B-cell lymphoma in relationship to mandatory WHO classification since 2008 and the next development of knowledge in this field with potential new influence on an advancement of both classification and therapy.
Asunto(s)
Linfoma de Células B Grandes Difuso/clasificación , Humanos , Linfoma de Células B Grandes Difuso/patología , Linfoma de Células B Grandes Difuso/terapia , PronósticoRESUMEN
BACKGROUND: Symptomatic cardiac involvement is the most important prognostic factor in AL amyloidosis patients. Longterm survival is limited not only by cardiac involvement condition, but also by limited choice of treatment with unsatisfactory results. The aim of the present report is to assess the effect of achieved treatment response on survival of AL amyloidosis patients with symptomatic cardiac involvement under conventional treatment. MATERIAL AND METHODS: The monitored patient set consisted of 19 patients with systemic AL amyloidosis and symptomatic cardiac involvement, treated and monitored at the III. Clinic of Internal Medicine between 2004 and 2012. The male : female ratio was 17 : 2, and the age median was 64 (range 48 to 78 years). Thirteen patients died within the monitored period. Functional status was defined according to the NYHA classification, where five patients had class II involvement, 10 patients had class III involvement, and four patients had class IV involvement. Treatment response was assessed by the application of modified IMWG and ISA criteria; all patients were undergoing conventional treatment. Nine patients were treated by a combination of alkylating agents (alkeran, cyclophosphamide), six were treated by a combination treatment with thalidomide, and four were treated by a combination of bortezomib and dexamethasone. Data were analyzed with software SPSS v. 15 (SPSS, Inc., Chicago, USA). Log Rank Test was applied to survival evaluation. RESULTS: The statistical analysis included only 13 patients who underwent at least three months of treatment, where six patients attained complete remission (CR), four patients attained partial remission (PR), and three patients attained only stabilization of disease (SD). Significant difference in patient survival was found to be correlated with attained hematological response, where the patients who attained CR had median survival of 39 months vs 10 months in patients who attained PR or SD (p = 0.005). CONCLUSION: The results indicate that attainment of complete hematological remission is associated with significantly longer survival of AL amyloidosis patients with symptomatic cardiac involvement.
Asunto(s)
Amiloidosis/tratamiento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Dexametasona/administración & dosificación , Neoplasias Cardíacas/tratamiento farmacológico , Inducción de Remisión/métodos , Anciano , Amiloidosis/complicaciones , Amiloidosis/mortalidad , Ácidos Borónicos/administración & dosificación , Bortezomib , Ciclofosfamida/administración & dosificación , Femenino , Neoplasias Cardíacas/complicaciones , Neoplasias Cardíacas/mortalidad , Humanos , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas , Masculino , Melfalán/administración & dosificación , Persona de Mediana Edad , Pirazinas/administración & dosificación , Talidomida/administración & dosificación , Resultado del TratamientoRESUMEN
INTRODUCTION: Cardiac involvement is a dominant prognostic factor in AL amyloidosis patients. A detailed assessment of the presence and degree of cardiac involvement utilizes an array of noninvasive investigation methods, particularly echocardiography and MRI; laboratory parameters include troponins and natriuretic peptides. Cardiac involvement detection aside, cardiac bio-markers are used as a relatively strong stratification and prognostic factor. OBJECTIVE: The presentation of cardiac bio-markers assay applications in AL amyloidosis patients at an individual treatment center. PATIENTS AND METHODS: The monitored patient set consisted of 22 patients with histologically confirmed AL amyloidosis, of whom 18 met the criteria for cardiac involvement. Levels of cardiac bio-markers troponin T (TnT) and Nterminal probrain natriuretic peptide (NTâProBNP) were determined in all patients. Risk stratification of the patients utilized the Mayo staging system which is based on both bio-markers assays; Log Rank Test was applied to survival evaluation. RESULTS: Median survival of patients with cardiac involvement stigmata was 10 months vs 60 months survival of patients without signs of cardiac involvement (p = 0.133). Of the 4 patients without cardiac involvement, 1 has shown positive levels of TnT and 2 positive levels of NTâProBNP. All cardiac involvement patients exhibited abnormal levels of NTâProBNP (median 4,752 ng/âl; 415.7-â35,000) as well as positive levels of TnT (median 0.0815 µg/âl; 0.02-â0.986). The application of the Mayo stratification system to the set had determined 2 patients at stage I, 5 patients at stage II and 15 patients at stage III. The median survival of the Mayo I + II group vs the Mayo III group was 60 vs 6 months (p = 0.015), revealing extremely limited survival of stage III patients. Assessment of TnT and NTâProBNP levels relative to treatment response shows that the degree of decrease in both markers depends on maximum treatment response -â respectively the attainment of a complete hematological remission. CONCLUSION: The results, although obtained from a limited set of patients, confirm a definitive benefit of the application of cardiac bio-markers assay in the diagnostic and therapeutic algorithm of AL amyloidosis patients. The Mayo stratification system utilizing the cardiac indicator values represents a robust tool for risk stratification of AL amyloidosis patients.