RESUMEN
AIM: To study morphoimmunological and clinical features of primary mediastinal large B-cell lymphoma (PMLBCL). MATERIAL AND METHODS: We analysed the results of biopsy material study and treatment of 86 PMLBCL patients, effects of different factors on the disease prognosis, efficacy of some therapeutic programs and overall therapeutic efficacy. RESULTS: PMLBCL manifests mainly with massive lesions of anterior upper mediastinum with involvement of adjacent organs and tissues, absence of bone marrow involvement, frequent affection of CNS. Many primary patients have resistance to treatment, sensitive patients have no late recurrences. CONCLUSION: PMLBCL is an independent extranodal variant of non-Hodgkin's lymphoma with special clinical and morphoimmunological characteristics. Criteria are proposed for differential diagnosis of different variants of PMLBCL. New approaches to PMLBSL patients' management are outlined.
Asunto(s)
Linfoma de Células B Grandes Difuso/patología , Neoplasias del Mediastino/patología , Neoplasias del Timo/patología , Adolescente , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/administración & dosificación , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Supervivencia sin Enfermedad , Femenino , Humanos , Linfoma de Células B Grandes Difuso/tratamiento farmacológico , Linfoma de Células B Grandes Difuso/genética , Masculino , Neoplasias del Mediastino/tratamiento farmacológico , Neoplasias del Mediastino/genética , Persona de Mediana Edad , Neoplasias del Timo/tratamiento farmacológico , Neoplasias del Timo/genética , Adulto JovenRESUMEN
Epstein-Barr virus (EBV) is an etiological agent of a number of benign and malignant human diseases, such as infectious mononucleosis (IM), Hodgkin's lymphoma (HL), and non-Hodgkin's lymphoma (NHL). EBV latent membrane protein 1 (LMP1) gene (recognized as a viral oncoprotein) of various clinical and geographical origin was found to have different types of amino acid mutations affecting its biological activity. Since there was no information on the strain differences in LMP1 of EBV persisting in Russia, the authors made a sequence analysis of LMP1 samples amplified from the biological materials of Russian patients with IM, HL, and NHL and healthy individuals. The studies have shown that LMP1 variants of Russian origin are a mixed heterogeneous group containing both the earlier characterized and presumably new genetic variants. Among the point amino avid substitutions, the mutations S366T, F106Y, 185L, and E328Q associated with the enhanced transforming activity of a LMP1 molecule and its reduced cytotoxicity. There was no specific association between the certain Russian variants of LMP1 and the specific forms of the disease (IM, HL, and NHL).