Assuntos
Neoplasias das Glândulas Suprarrenais/metabolismo , Centro Germinativo/metabolismo , Linfoma Difuso de Grandes Células B/metabolismo , Proteínas Proto-Oncogênicas c-bcl-2/metabolismo , Neoplasias das Glândulas Suprarrenais/diagnóstico , Idoso , Feminino , Humanos , Linfoma Difuso de Grandes Células B/diagnóstico , Masculino , Pessoa de Meia-Idade , Fenótipo , PrognósticoRESUMO
A member of the family of p53-related genes, p63 plays a role in regulating epithelial proliferation and differentiation programs, but the pathological and clinical meaning of p63 in B-cell lymphoma has not been elucidated. We investigated the expression pattern of p63 in B-cell malignancies, and evaluated the correlation between the expression of p63 and other germinal center markers. Ninety-eight B-cell lymphomas (28 FCL, 5 MCL, and 65 DLBCL) were analyzed by immunohistochemical examination for p63, bcl-6, CD10 and MUM-1 proteins, and for rearrangement of bcl-2/IgH. Expression of p63 was observed in the nuclei of tumor cells obtained from 15 of 28 (54%) FCL, 22 of 65 (34%) DLBCL, but none of 5 MCL. In DLBCL, the expression of p63 and bcl-6 showed a significant correlation (P < 0.02), but no correlation was observed between p63 and expression of CD10, MUM-1, or bcl-2/IgH rearrangement. RT-PCR revealed that TAp63alpha-type transcripts, a possible negative regulator of transcriptional activation of p21 promoter, were major transcripts in B-cell lymphoma tissues. As for prognostic significance, only patients in the p63 positive group of FCL died, and in the non-germinal center group, the p63 positive cases appeared to have inferior overall survival than other groups in DLBCL. Our preliminary results suggested that p63 expression is a disadvantageous factor for prognosis in this subgroup of B-cell lymphomas.
Assuntos
Proteínas de Ligação a DNA/análise , Linfoma de Células B/diagnóstico , Transativadores/análise , Proteínas Supressoras de Tumor/análise , Adulto , Idoso , Idoso de 80 Anos ou mais , Processamento Alternativo , Proteínas de Ligação a DNA/genética , Feminino , Humanos , Linfoma de Células B/mortalidade , Linfoma de Células B/patologia , Masculino , Pessoa de Meia-Idade , Prognóstico , RNA Mensageiro/análise , Transativadores/genética , Fatores de Transcrição , Proteínas Supressoras de Tumor/genéticaRESUMO
An 83-year-old man without history of the hemorrhagic diathesis was admitted to our hospital with a 4-months history of purpura and subcutaneous hematoma. He had an extraordinarily prolonged activated partial thromboplastin time, and his factor VIII (F VIII) activity level was 0.2%. A study revealed the existence of an IgG type anti-F VIII inhibitor at a titer of 1004 Bethesda units/ml. He received recombinant factor VIIa and immunosuppressive therapy with cyclophosphamide, prednisolone and cyclosporin, but despite this the titer of F VIII inhibitor remained high. Although the inhibitor disappeared after methylprednisolone mini-pulse therapy, the patient died of opportunistic infections with cytomegalovirus and pneumocystis carinii. The majority of patients with acquired F VIII inhibitor belong to the elderly population, and the standard therapeutic strategy to eliminate the acquired F VIII inhibitor has not been established. Those patients with high titers of F VIII inhibitor require particularly long term immunosuppressive therapy. Therefore, it is important to bear in mind treatment-related opportunistic infections in a case with a high titer of acquired F VIII inhibitor.