RESUMO
Although there is a worldwide interest in the assessment of health-related quality-of-life (HRQoL) in haemophilia patients, no non-disease specific instruments (for adults) are readily available. In this paper, a haemophilia-specific quality-of-life assessment measure for adults (the Hemofilia-QoL questionnaire) has been developed and tested for psychometric properties in 121 adults with haemophilia living in Spain. The Hemofilia-QoL questionnaire is a self-report modular instrument that assesses nine relevant HRQoL domains for patients with haemophilia (e.g. physical health, daily activities, joint damage, pain, treatment satisfaction, treatment difficulties, emotional functioning, mental health, relationships and social activity). Psychometric examination involved the assessment of data quality, scaling assumptions, reliability (internal consistency and test-retest) and validity (concurrent; external clinical criterion and sensitivity). The Hemofilia-QoL 36-item version questionnaire had acceptable internal consistency and retest reliability values. The questionnaire shows excellent concurrent validity (with the SF-36 Health Survey) and external clinical criterion validity (haemophilia clinical status) and sensitivity (health status changes) as well. The Hemofilia-QoL is now available for adult assessment and is ready for use in clinical research in Spain.
Assuntos
Hemofilia A/reabilitação , Qualidade de Vida , Adolescente , Adulto , Idoso , Estudos Transversais , Nível de Saúde , Hemartrose/etiologia , Hemofilia A/complicações , Hemorragia/etiologia , Humanos , Pessoa de Meia-Idade , Dor/etiologia , Satisfação do Paciente , Psicometria , Reprodutibilidade dos Testes , Inquéritos e Questionários , Viroses/etiologiaRESUMO
A haemophilia-specific health-related quality-of-life questionnaire (named 'Hemofilia-QoL') was developed to assess quality-of-life in adults with haemophilia, and was psychometrically tested. Seventy-three interviews with haemophilia patients and health care professionals were used to generate the items included in the questionnaire, and expert ratings on the items formulated were used to screen them for potential omission. This was followed by psychometric testing in a sample of 35 patients. Preliminary psychometric testing of the revised questionnaire version, which contains 10 domains (physical health, physical role, joint damage, pain, treatment satisfaction, emotional role, mental health, social support), showed acceptable reliability (alpha = 0.94 for the Hemofilia-QoL total score) and validity, and this will be examined in a subsequent study with a larger patient sample.
Assuntos
Hemofilia A/psicologia , Qualidade de Vida , Inquéritos e Questionários/normas , Adolescente , Adulto , Humanos , Pessoa de Meia-Idade , Projetos Piloto , PsicometriaRESUMO
Inasmuch as Sudan black B stain is highly specific for myeloid cells and since over 3% positive blast cells meet the needs of diagnosis for myeloblastic leukaemias, we had the opportunity to study a 27 year-old male with acute leukaemia whose morphological, cytochemical, immunocytochemical (HLA-DR, CD-10, CD-19, CD-20, CD-21 all positive) and ultrastructural features were clearly in accordance with acute lymphoblastic leukaemia, except for 42% Sudan black B positivity of the bone marrow blast cells. The practical interest of this case comes from the necessity to take this rare ALL case into account.
