RESUMO
Background: Hodgkin lymphoma is a highly curable disease. Aim: To evaluate the clinical characteristics and the treatment results of Hodgkin lymphoma patients of the National Cancer Program in Chile. Patients and methods: Prospective assessment of 682 patients treated in 18 adult cancer centers. Progression free survival (PFS) and overall survival (OS) were calculated. Median follow up was 127, 95, 87, 72 and 50 months for C-MOPP, radiotherapy (RT), C-MOPP/ABV, NOVP and ABVD, respectively. Results: Median age was 37 years (15-84). Nodular sclerosis and mixed cellularity were equally expressed. Advanced stages (III & IV) were present at diagnosis in 61 percent of cases. Age over 40 was an adverse prognostic factor (p <0.001). The rate of PFS at 5 and 10 years for early stages was 73 percent and 66 percent with RT, 80 percent and 74 percent with C-MOPP+RT, 73 percent and 71 percent with C-MOPP/ABV, 59 percent and 59 percent with NOVP+RT, and 81 percent with ABVD+RT, at 5 years, being significantly lower for NOVP (p =0.02). The rate of OS at 5 and 10 years for advanced stages was 82 percent and 70 percent with RT, 82 percent and 76 percent with C-MOPP+RT, 82 percent and 80 percent with C-MOPP/ABV, 68 percent and 60 percent with NOVP, and 85 percent with ABVD at 5 years, also significantly lower for NOVP (p =0.04). For advanced stages, the rate of PFS at 5 and 10 years was 49 percent and 43 percent with C-MOPP, 69 percent and 62 percent with C-MOPP/ABVD or C-MOPP/ABV, and 71 percent at 5 years with ABVD, significantly lower for C-MOPP (p =0.01). The rate of OS at 5 and 10 years was 52 percent and 46 percent with C-MOPP, 70 percent and 63 percent with C-MOPP/ABVD or C-MOPP/ABV and 76 percent with ABVD at 5 years, significantly lower for C-MOPP (p =0.0002). Conclusions: Age over 40 years was an adverse prognostic factor. C-MOPP/ABVD, C-MOPP/ABV and ABVD had comparable results and reached a high tumor control and overall survival in both early...
Assuntos
Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Doença de Hodgkin/tratamento farmacológico , Programas Nacionais de Saúde , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Bleomicina/administração & dosagem , Distribuição de Qui-Quadrado , Chile , Ciclofosfamida/administração & dosagem , Dacarbazina/administração & dosagem , Intervalo Livre de Doença , Doxorrubicina/administração & dosagem , Seguimentos , Doença de Hodgkin/radioterapia , Mitoxantrona/administração & dosagem , Prednisolona/administração & dosagem , Prednisona/administração & dosagem , Procarbazina/administração & dosagem , Estudos Prospectivos , Resultado do Tratamento , Vimblastina/administração & dosagem , Vincristina/administração & dosagemRESUMO
Background: Adult T cell leukemia lymphoma is a lymphoproliferative syndrome etiologically associated to human T cell lymphotropic virus type I. Aim: To describe the clinical and laboratory features of 26 caucasian chilean patients, with HTLV-I positive adult T-cell leukemia lymphoma (ATLL). Material and methods: Diagnostic criteria included clinical features, cell morphology, immunophenotype, HTLV-I serology and/or DNA analysis by southern blot or PCR. Results: According to the clinical presentation, 12 cases had the acute ATLL form, 6 had a lymphoma, 4 the chronic form and 4 had smoldering ATLL. The median presentation age was 50 years, younger than the Japanese patients, but significantly older than patients from other south american countries (eg Brasil, Jamaica, Colombia). The main clinical features: lymphadenopathy, skin lesions and hepatosplenomegaly, were similar in frequency to those of patients from other countries, except for the high incidence of associated neurological disease. Tropical spastic paraparesis (TSP) in our series of ATLL, was seen in one third of the patients (8/26). A T-cell immunophenotype was shown in all 26 cases and HTLV-I serology was positive in 25/26 patients. Molecular analysis on the seronegative patient showed clonal integration of proviral HTLV-I DNA into the lymphocytes DNA, and thus he may have been a poor responder to the retroviral infection. Proviral DNA integration was also demonstrated in 15/16 patients being clonal in 10, polyclonal in 3 (all smoldering cases) and oligoclonal in one. Conclusions: ATLL in Chile has similar clinical and laboratory features than the disease in other parts of the world, except for a younger age than japanese patients but older than those from other latin american countries and a high incidence of patients with associated TSP. Detailed morphological and immunophenotypic analysis of the abnormal circulating lymphocytes, together with the documentation of HTLV-I by serology and/or DNA analysis are key tests for the identification of this disease