Determining the health-related quality of life in individuals with haemophilia in developing economies: results from the Brazilian population.

INTRODUCTION: Several studies show the negative impact of haemophilia in health-related quality of life (HRQOL). This issue is not well explored in developing countries. OBJECTIVES: This cross-sectional study aimed to evaluate the HRQOL and its associated factors in patients with haemophilia A/B in Brazil. Data were collected by questionnaire and in medical records, including a Portuguese version of Haem-A-Qol. RESULTS: Brazilian patients were invited to the study and 175 participants (147 haemophilics A and 28 haemophilics B) were included. The total score of the Haem-A-QoL had a median of 36.96 (range of 0-100), with worse performance in 'sport and leisure' and best on 'relationships' fields. HRQOL was worst among the older participants, the less educated, non-white, non-working, who were hospitalized in the last year, who did not have a single medical consultation and among those with the highest number of affected joints. Moreover, patients with hepatitis B had a significantly worse HRQOL in the domain 'sports and leisure', also observed in married patients. Otherwise, married individuals reported better HRQOL on 'dealing with the disease' domain. Patients with haemophilia B reported worse HRQOL in the domain 'self-perception'. CONCLUSION: The results obtained could be helpful in guidance of haemophilia treatment which is determinant to improve HRQoL of the most vulnerable groups of patients. This work also reinforced the relevance of joint bleeds in all aspects of HRQoL in haemophilic patients. The use of prophylactic factor concentrates and multidisciplinary treatments could contribute to improve the quality of life in haemophilia.

Neoplasias múltiplas após tratamento bem sucedido de doença de Hodgkin / Multiple neoplasias after a well succeeded treatment of Hodgkin's disease

Paciente do sexo feminino que em 1977, aos 21 anos, apresentava febre persistente e linfadenomegalia cervical e axilar, cuja biópsia revelou doença de Hodgkin, tipo esclerose nodular, estádio IIIB. A paciente recebeu seis ciclos de quimioterapia associados a imunoterapia e suplementada por radioterapia, com boa evoluçäo. RESULTADOS. Em 1985, durante rotina ginecológica e histerctomia posterior, foram dignosticados neoplasia escamosa intra-epitelial cervical grau III (NIC III) e leiomioma atípico do corpo uterino. Cinco anos mais tarde, aos 34 anos, desenvolveu carcinoma ductal com metaplasia cartilaginosa de mama direita com metástase para linfonodos cervicais e axilares. A paciente foi submetida a tratamento quimio e radioterápico, evoluindo para óbito nove meses mais tarde. CONCLUSAO. A possibilidade da ocorrência tardia de neoplasias em pacientes submetidas a tratamento de doença de Hodkin sugere seu seguimento mais rigoso e continuado, até mesmo com o emprego de mamografias periódicas

[Multiple neoplasms after a well succeeded treatment of Hodgkin's disease]. / Neoplasias múltiplas após tratamento bem sucedido de doença de Hodgkin.

The authors refer to a 21-year-old Caucasian (white) woman, who in 1977 presented fever and cervical and axillary adenopathy, whose biopsy showed nodular sclerosis Hodgkin's Disease, stage IIIB. The patient received six chemotherapy cycles associated with immunotherapy and supplemented with radiation therapy with good response. RESULTS--In 1985, after routine gynaecological examination and a hysterectomy, cervical intraepithelial neoplasia grade 3 (CIN 3) and atypic leiomyoma of the uterine body were diagnosed. Five years later, biopsies diagnosed invasive duct carcinoma in the right breast and homolateral axillary and cervical nodes. The patient was submitted to chemo and radiation therapy and died nine months later. CONCLUSION--The possibility of later occurrence of a second or multiple new malignancies in patients successfully treated for Hodgkin's Disease points out the need for a more complete long-term follow-up, including periodic mammography.