RESUMO
Hereditary prothrombin deficiency is an autosomal recessive disorder with an estimated incidence of 1 in 2 million . Presentation of the disease is variable; however, it is usually associated with moderate to severe bleeding tendencies including muscle hematomas, hemarthrosis, intracranial, mucosal, and postoperative bleeding. Here we report a case of a 35-year-old pregnant woman with congenital hypoprothrombinemia and idiopathic thrombocytopenic purpura, review the literature, and discuss its epidemiology, presentation, diagnosis, and treatment.
Assuntos
Transtornos Hemorrágicos/complicações , Hipoprotrombinemias/complicações , Púrpura Trombocitopênica Idiopática/complicações , Adulto , Feminino , Humanos , GravidezRESUMO
BACKGROUND: Evans syndrome (ES) is characterized by the simultaneous or sequential presence of multiple autoimmune cytopenias. It is often secondary to rheumatologic disorders or lymphoid malignancies, but has not previously been associated with babesiosis. Here we present two cases of severe cytopenias in asplenic patients precipitated by active babesiosis. CASE REPORT: The first patient had a history of Hodgkin's lymphoma in remission and autoimmune hemolytic anemia (AIHA) treated by splenectomy 12 years prior who presented with severe AIHA and thrombocytopenia after Babesia infection. The second patient had a history of ES requiring splenectomy, which relapsed after Babesia infection. RESULTS: The complex presentation and medical histories of both patients made the diagnosis challenging. Both patients' cytopenias responded to therapy, although the use of immunosuppressive agents in patients with active hematologic infections was challenging and required a multidisciplinary approach. CONCLUSION: These two cases illustrate the possibility of babesiosis to not only reactivate ES in asplenic patients, but also precipitate increased levels of immune deregulation, potentially provoking ES, a phenomenon not previously reported.
Assuntos
Anemia Hemolítica Autoimune/etiologia , Babesiose/complicações , Parasitemia/complicações , Trombocitopenia/etiologia , Adulto , Anemia Hemolítica Autoimune/cirurgia , Babesiose/diagnóstico , Transfusão de Sangue , Feminino , Doença de Hodgkin/complicações , Humanos , Hospedeiro Imunocomprometido , Masculino , Pessoa de Meia-Idade , Parasitemia/diagnóstico , Indução de Remissão , Esplenectomia/efeitos adversos , Trombocitopenia/cirurgiaRESUMO
Leiomyosarcoma of the rectum can develop as a late complication in patients with a history of pelvic irradiation. We report the case of a patient who developed rectal leiomyosarcoma 13â years after receiving radiation for treatment of a stage 2 squamous cell cancer of the anus. This was detected on physical examination. Based on a discussion with the patient, we decided to manage conservatively. Overall, leiomyosarcomas constitute 5-12% of radiation-induced sarcomas. Rectal leiomyosarcoma is rare, accounting for 0.1-0.5% of all malignant tumours of the rectum. As radiation therapy plays a major role in the management of anal cancer, it is important that clinicians are aware of the possible development of radiation-induced sarcomas that may occur decades after initial management.