RESUMO
Glanzmanns thrombasthenia (GT) is a rare bleeding syndrome characterized by deficiency or defect of platelet aggregation complex. The pathogenesis of endometriosis is controversial but the strongest evidence leans towards retrograde menstruation. GT probably predisposes to endometriosis. The management of women affected by this disease can be difficult due to the risk of bleeding complications, especially during surgical treatment. We describe the cases of three sisters affected by endometriosis and GT, referred to our Department, who received different therapeutic management.
Assuntos
Endometriose/etiologia , Trombastenia/complicações , Adulto , Anticoncepcionais Orais Hormonais/efeitos adversos , Anticoncepcionais Orais Hormonais/uso terapêutico , Suscetibilidade a Doenças , Doenças em Gêmeos , Endometriose/diagnóstico por imagem , Endometriose/tratamento farmacológico , Endometriose/cirurgia , Fator VIIa/uso terapêutico , Feminino , Hematometra/etiologia , Transtornos Hemorrágicos/tratamento farmacológico , Transtornos Hemorrágicos/etiologia , Humanos , Dispositivos Intrauterinos Medicados , Levanogestrel/uso terapêutico , Acetato de Medroxiprogesterona/uso terapêutico , Menorragia/etiologia , Doenças Ovarianas/diagnóstico por imagem , Doenças Ovarianas/tratamento farmacológico , Doenças Ovarianas/etiologia , Doenças Ovarianas/cirurgia , Assistência Perioperatória , Proteínas Recombinantes/uso terapêutico , Doenças Retais/diagnóstico por imagem , Doenças Retais/tratamento farmacológico , Doenças Retais/etiologia , Trombastenia/genética , Ácido Tranexâmico/uso terapêutico , Pamoato de Triptorrelina/uso terapêutico , Doenças Vaginais/diagnóstico por imagem , Doenças Vaginais/tratamento farmacológico , Doenças Vaginais/etiologiaRESUMO
Ovarian cancer is burdened by the highest mortality rate among gynecological cancers. Gold standard is represented by the association of platinum-taxane -based chemotherapy and radical surgery. Despite several adjustments occurred in cytotoxic drug in last decades, most patients continue to relapse, and no significant enhancement has been reached in the overall survival. The development of drug resistance and the recurrence of disease have prompted the investigations of other targets that can be used in the treatment of ovarian cancers. Among such targets, polyadenosine diphosphate-ribose polymerase (PARP) represents a novel way to target specific patways involved in tumor growth. PARP accelerates the reaction of the polyADP-ribosylation of proteins implicated in DNA repair. PARP inhibitors have shown activity in cancers with BRCA mutations, with other deficient DNA repair genes or signaling pathways that modulate DNA repair, or in association with DNA damaging agents not involved in DNA repair dysfunction. A number of inhibitors for PARP has been developed, and such drugs are under investigation in clinical trials to identify their impact in the treatment of ovarian cancers. This review aims to summarize the recent researches and clinical progress on PARP inhibitors as novel target agents in ovarian cancer.