RESUMO
PURPOSE: To compare the clinical patterns and survival of young and adult (AP) versus paediatric (PP) patients with paroxysmal nocturnal haemoglobinuria (PNH). PATIENTS AND METHODS: The clinical records of 117 patients (82% AP, 18% PP) seen in four cities of the Mexican Republic were analysed, the clinical course and survival of both groups being compared. RESULTS: No sex difference was found in the two patient-groups: 51% and 52% males, 49% and 48% females in AP and PP, respectively. The onset of PNH had similar distribution for the two groups of patients: aplastic form, 45% in AP and 62% in PP; cytopenias, 24% in AP versus 27% in PP; haemolysis, 28% in AP and 9% in PP, and thrombosis, 3% in AP versus 0% in PP. The clinical features with significant difference were: anaemic+haemorrhagic syndrome (39 AP (40%) vs 14 PP (67%), p = 0.02), initial diagnosis of immunologic thrombocytopenic purpura (7 AP (7%) vs 7 PP (33%), p = 0.003), and death rate (17 AP (18%) vs 8 PP (38%), p = 0.04). The actuarial survival curves showed significant differences between both groups (p = 0.045, Cox-Mantel), with estimated 10-year survival of 81% for AP and 55% for PP, and 15-year survivals of 64% for AP and 55% for PP. CONCLUSIONS: Seemingly, PNH in paediatric age has poorer prognosis than in adults, which is associated to higher incidence of fatal haemorrhages due to thrombocytopenia.
Assuntos
Hemoglobinúria Paroxística/epidemiologia , Doença Aguda , Adolescente , Adulto , Anemia/etiologia , Anemia Refratária com Excesso de Blastos/etiologia , Medula Óssea/patologia , Criança , Pré-Escolar , Diagnóstico Diferencial , Feminino , Hemoglobinúria Paroxística/classificação , Hemoglobinúria Paroxística/complicações , Hemoglobinúria Paroxística/patologia , Hemorragia/etiologia , Humanos , Incidência , Lactente , Leucemia Mieloide/etiologia , Tábuas de Vida , Masculino , México/epidemiologia , Prognóstico , Púrpura Trombocitopênica Idiopática/diagnóstico , Estudos Retrospectivos , Análise de Sobrevida , Trombose/etiologiaRESUMO
Se presentan nueve casos de tumor de Warthin afectando a la glándula parótida, estudiados desde un punto de vista clínico, radiológico e histopatológico. Se discuten los aspectos más interesantes en el diagnóstico de esta entidad. En todos ellos se practicó sialografía y estudio radioisotópico. El seguimiento medio efectuado fue de dos años y nueve meses tras el tratamiento quirúrgico, que se practicó en todos los casos, no existiendo recidiva alguna. Tan sólo en un caso se apreció paresia del facial superior homolateral, que desapareció posteriormente con tratamiento médico