RESUMO
OBJECTIVES: This study aims to identify the presenting symptoms, treatment and outcome of patients with nasal natural killer T (NK/T)-cell lymphoma and to find possible differences in survival based on Ann-Arbor stage and international prognostic index (IPI). PATIENTS AND METHODS: Computed tomography and biopsy results of 23 patients (15 males, 8 females; mean age 41 years; range 22 to 72 years) with extranodal NK/T-cell lymphoma who were treated at the department of clinical hematology between 1995 and 2011 were retrospectively analyzed. RESULTS: The median time from onset of clinical symptoms to histological diagnosis was five months. Most patients presented with nasal obstruction (69%) and rhinism (52%). The site of extranodal NK/T-cell lymphoma primarily involved nasal cavity in 39%. Orbital extension was observed in 26%. Lymphomas were classified as stage IE in 30.4%, stage IIE in 47.8% and stage IVE in 21.7%. Nineteen patients received treatment: 10 received chemotherapy plus radiotherapy, nine received chemotherapy only. We used several regimens of chemotherapy including some protocols containing etoposid, L-asparaginase and others without this drugs. Univariate analysis showed that lower IPI score, low Ann-Arbor stage and responsiveness to treatment with both chemotherapy and radiotherapy were significant factors influencing both OS and PFS. CONCLUSION: Nasal type NK/T-cell lymphoma showed a poor response to the conventional anthracycline-based chemotherapy, thereby an investigation for a novel therapy is urgently needed to improve survival.
Assuntos
Linfoma de Células T/mortalidade , Linfoma de Células T/terapia , Neoplasias Nasofaríngeas/mortalidade , Neoplasias Nasofaríngeas/terapia , Neoplasias Nasais/mortalidade , Neoplasias Nasais/terapia , Adulto , Idoso , Feminino , Humanos , Linfoma de Células T/diagnóstico , Masculino , Pessoa de Meia-Idade , Neoplasias Nasofaríngeas/diagnóstico , Células T Matadoras Naturais , Estadiamento de Neoplasias , Neoplasias Nasais/diagnóstico , Prognóstico , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento , Adulto JovemRESUMO
The association of essential thrombocythemia and multiple myeloma is extremely rare, with only three patients previously treated with hydroxyurea reported in the literature until now. In this paper, we report the case of a 66 year old male who developed IgG-kappa M six years after the diagnosis of essential thrombocythemia, for which he had received hydroyurea. The possible etiological and pathogenic link between both these entities is here discussed.