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R Grace

Publications and source records attributed to R Grace.

At least 19 recordsLinked to original sources

Cardiac presentation of ALK positive anaplastic large cell lymphoma.

Cardiac involvement as an initial presentation of malignant lymphoma is a rare occurrence. We report the case of an immunocompetent 29-year-old male who presented with syncope and arrythmias secondary to a ventricular cardiac mass. Transcutaneous cardiac biopsy was non-diagnostic, therefore an open cardiac biopsy was performed from which a provisional diagnosis of a cardiac inflammatory pseudotumour was made. Six months after presentation, he developed several subcutaneous lesions with systemic symptoms. Histological and immunophenotypic review of the initial cardiac biopsy revealed features consistent with a diagnosis of CD30, ALK1 positive anaplastic large cell lymphoma (ALCL). Despite intensive treatment with combination chemotherapy, there was significant progression of disease, and he died 11 months after diagnosis. The overall prognosis of cardiac lymphoma remains poor, which may be due to the often late presentation of the tumour. To our knowledge, this is the first reported case of a cardiac ALK positive ALCL. Although rare, cardiac presentation of ALCL should be added to the list of differential diagnoses of cardiac lymphomas.

Activin Receptors, Type I↗

Family factors influencing out-of-home placement decisions.

Flying in the face of national community care policies, families of young children with severe disabilities continue to seek out-of-home placement The present paper explores the factors which influence families to care for their children at home or to place them out-of-home. Data for the present study were derived from a qualitative in-depth study of the everyday family life experiences of 167 families of young children with a disability and high support needs. One hundred and twenty-five (75%) of these families definitely did not want to place their child, 32 (19%) were undecided, and 10 (6%) were actively seeking or had already sought placement. Coded interview data were subjected to exploratory factor analysis to reveal eight factors influencing everyday family life. An analysis of variance (ANOVA) revealed significant differences between the three groups of families on three out of the eight factors. The families' views about placing their child were compared across the three groups using text analysis techniques. Without exception, the primary desire of all families was to care for their child at home. However, when placement was considered a possibility, even if remote, the most frequently reported reasons were family 'survival' and mitigating circumstances. The finding that one-quarter of the families had already sought or were considering placement for children in this young age range is provocative. The implications of the findings for policy and practice are discussed.

Child↗

Fludarabine, cytarabine, G-CSF and idarubicin (FLAG-IDA) for the treatment of poor-risk myelodysplastic syndromes and acute myeloid leukaemia.

Nineteen patients with high-risk myelodysplastic syndrome (MDS)/acute myeloid leukaemia (AML) received fludarabine, cytarabine, granulocyte-colony stimulating factor (G-CSF), and idarubicin chemotherapy (de novo MDS/MDS-AML, nine; relapsed/refractory MDS/AML, seven; therapy-related MDS, three). Median age was 44 years and median disease duration 10 months. 16/19 (84%) patients had abnormal cytogenetics with seven (37%) harbouring abnormalities of chromosome 7. 18/19 (94.7%) patients responded to FLAG-idarubicin with 12 (63%) achieving complete remission (CR) (< 5% blasts and normal cytogenetics). 7/9 (78%) patients with de novo MDS/MDS-AML achieved CR compared to 5/10 (50%) with alternative diagnoses. Response was associated with age < 50 years, disease duration < 3 months, and cytogenetics other than abnormalities of chromosome 7. Haemopoietic regeneration was rapid in most patients and there were no toxic deaths. Nine patients received a second course of chemotherapy, three have proceeded to allogeneic bone marrow transplant and three to autologous blood stem cell/bone marrow transplantation. Follow-up is short (median 10 months). 12/19 (63%) patients remain alive and 5/12 (42%) have relapsed at a median 5 months following CR achievement. FLAG-idarubicin was well tolerated. High rates of morphological and cytogenetic remission, especially in de novo MDS, offer a window of opportunity for assessment of autologous BMT in this group of diseases where no treatment except alloBMT has led to prolongation of survival.

Acute Disease↗