The prolonged survival of patients with thalassemia major as a result of the novel therapeutic strategies introduced in the last decade makes patient quality of life an important issue. This study ...investigated the changes occurring in overall quality of life in patients with thalassemia in the last decade.
This was a population-based cross-sectional survey of quality of life in the entire population with thalassemia major resident in the Liguria region of Italy from 2001 to 2009. The self-administered Short Form-36 (SF-36) questionnaire was used to measure quality of life in patients with thalassemia.
Forty-nine and 52 eligible patients were assessed in 2001 and 2009, respectively. A total of 43 patients were assessed in both 2001 and 2009. Almost 40% of these 43 patients received deferasirox in 2009, a drug which was not available in 2001. The distribution of ferritin levels was lower in 2009 (median 730) as compared with 2001 (median 1107). Analysis of the raw differences between the two years did not show a significant difference. An improvement was observed in most SF-36 scales in 2009 as compared with 2001, particularly in the Mental Health scale (mean difference in Z score +4.0; 95% confidence interval 0.4-7.5; P = 0.030) and in the Mental Component Summary scale (mean difference in Z score +3.2; 95% confidence interval 0.2-6.2; P = 0.039).
The challenge associated with new therapies and improvement in mental quality of life dimensions indicates that implementation of effective interventions for screening and evaluation of quality of life is now urgent.
Forty-three consecutive patients with de novo and untreated non M3 AML aged 60 or less entered the study. The mean age of patients was 50 (range 15-60). The induction regimen (FLAG-Ida) included ...fludarabine (30 mg/sqm), Ara-C (2 g/sqm) on days 1-5, and idarubicin (10 mg/sqm) on days 1, 3, 5. G-CSF (300 mcg/day) was administered s.c. 12 hours before starting fludarabine and was continued for five days. HDT with stem cell rescue was planned for all patients in first CR after one course of high dose Ara-C (HDAC) consolidation and in good clinical conditions. Forty-two (98%) patients were evaluable for response. One patient died during induction (2%). CR was achieved in 35 patients (82%). Twenty-three patients, 66% of those achieving CR, underwent autologous (N = 17) or allogeneic (N = 6) transplantation. With a median follow up of 24 months, the average median duration of CR is 17 months (range 3-66) and the median survival is 20 months (range 1-83). Overall the 5 year projected disease free survival (DFS) and overall survival (OS) were 37% and 43%, respectively. Among patients who underwent stem cell transplantation DFS and OS were 53% and 69%, respectively. The median time to PMN recovery (> 0.5 x 10(9)/l) was 17 days (range 10-28) and 50 x 10(9)/l platelets were reached at a median of 17 days (12-38). In conclusion FLAG-Ida regimen is effective, low toxic and improves feasibility of stem cell transplant.
Lymphoplasmacytic-lymphoplasmacytoid lymphoma (LPL)/Waldenstrom's macroglobulinemia (WM) or immunocytoma (IMC) consists of diffuse proliferation of small mature B lymphocytes, plasmacytoid ...lymphocytes, and plasma-cells. The nosographic definition includes the lack of histological, immunophenotypic, cytogenetic, and molecular markers considered specific of other types of lymphoma. The cells show surface Ig (usually IgM), B-cell-associated antigens and display the CD5-, CD23- and CD10- phenotype, which allows for differential diagnosis from B-CLL and mantle cell lymphoma. t(9;14)(p13;q32) chromosomal translocation has been found in 50% of all LPL cases. The cytogenetic rearrangement juxtaposes the PAX-5 gene, which encodes for an essential transcription factor for B-cell proliferation and differention, to the Ig heavy chain gene. The combination of chlorambucil and prednisone holds as the standard treatment and seems to guarantee good control of the disease in most patients. Similar therapeutic results have been described with the combination of cyclophosphamide, vincristine, prednisone with (CHOP) or without doxorubicin (CVP), or with a combination of other alkylating agents and prednisone. Nucleoside analogues, alone or in combination with alkylating agents and anthracyclines, provide good salvage therapy for IMC and being increasingly employed as first line therapy. In a multicentric European trial Foran et al. administered the chimeric anti-CD20-monoclonal antibody (Rituximab) to 28 patients with previously treated IMC. Seven out of 25 evaluable patients (28%) achieved a partial response. Byrd et al. examined the outcome of 7 previously treated WM patients who received weekly infusions of rituximab (375 mg/m2). Therapy was well tolerated by all patients, and there was no decrease in cellular immune function, or significant infectious morbidity. Partial responses were noted in three of these patients, including two with fludarabine-refractory disease. These data suggest that rituximab exerts clinical activity on heavily pre-treated patients with WM. Furthermore, Weide et al. first reported that WM-associated polyneuropathy can be treated effectively with a combination of chemotherapy and the anti-CD20 monoclonal antibody rituximab. Most published trials exploring the efficacy of high dose treatment as salvage therapy for relapsed or refractory low grade non Hodgkin's lymphoma have included prevalently follicular or lymphocytic lymphomas. In selected high risk patients radioimmunotherapy with autologous stem-cell rescue, and myeloablative therapy followed either by autologous stem cell transplantation (SCT) or allogeneic SCT might represent an alternative strategy.
A collaboration between the Observatoire de la Côte d'Azur (OCA) and the Centre National d'Études Spatiales (CNES) started in 1996 for detecting optically faint sources close to the geostationary ...ring using the OCA Schmidt camera. After a first approach based on photographic films we have installed a 2048 × 2048 CCD camera at the focus of the telescope. An automated analysis method was developed. After identifying all stellar trails on the CCD frames we substract them after a suitable modelization. The detection of the remaining objects takes into account the noise variations due to the model defects. For each CCD frame about two hundred point-like objects are identified. The detection of possible space objects is achieved by matching the lists of candidates obtained on three or more successive images. Few objects are observed at a limiting magnitude around 20. We determine their approximate orbits in order to allow follow up observations for each object. A software allowing the user to process the images just after the acquisition has been developed. A one year observational campaign is foreseen in the near future.
Sixty-two patients with high-risk acute leukemia were treated with the FLAD regimen 3 days of treatment with fludarabine 30 mg/m(2), cytarabine (AraC) 2 g/m(2), and liposomal daunorubicin 80 mg/m(2). ...The acute myeloid leukemia (AML) patients were either refractory to standard induction regimens (8), were in first or second relapse (13), or received therapy as first-line treatment 21 patients, 16 were above 60 years of age and 5 had post-myelodysplastic syndrome (MDS) AML. The acute lymphoblastic leukemia (ALL) patients were treated for relapsed (7) or refractory disease (10). Three patients had chronic myeloid leukemia (CML) in the blastic phase. FLAD was well tolerated by most patients. Ten major infectious complications were recorded while no signs of cardiac toxicity were observed. Five patients (8%) died before day 28 with hypocellular marrow, mainly of infection or hemorrhage, and response could not be evaluated. Complete response rate was 62% and 69% among AML patients treated at diagnosis or for relapsed disease, respectively, and 59% among the ALL patients. Furthermore, FLAD managed to overcome the negative impact of poor prognosis karyotype in ALL patients, since five of the seven patients with t(9;22) or complex karyotype achieved complete remission (CR). Nine patients underwent bone marrow transplantation (BMT). Among the AML patients who were treated at diagnosis or for relapse, the median duration of CR was 7 months (range: 2-18) and 8 months (range: 2-26), respectively. Median survival among these patients was 8 (range: 1-40) and 12 (range: 1-30) months, respectively. Similar values were found in ALL patients. In conclusion, FLAD may be an effective alternative treatment for patients with relapsed AML and for patients with ALL who failed first-line therapy.
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Objective: In this prospective study we evaluate the effects of high‐dose recombinant human erythropoietin (rHuEPO) on quality of life (QOL) and brain function in patients with low‐risk ...myelodysplastic syndromes (MDS) (<10% marrow blasts). Preliminary data are reported.
Methods: Eleven consecutive patients were given rHuEPO (40 000 IU two times a week) for 12 wk. Responsive patients continued with 40 000 IU/wk for further 12 wk. Changes in QOL were assessed by the Functional Assessment of Cancer Therapy‐Anemia (FACT‐An) self‐report. Neurophysiological evaluation at the start of the therapy (t0) included duplex scanning of neck vessels, transcranial Doppler sonography (TCD), a complex neuropsychological evaluation, and quantitative electroencephalography (qEEG). Eight patients completed the neurophysiological evaluation after 24 wk (t1).
Results: Six patients (55%) achieved an erythroid response after 12 wk, which was maintained after 24 wk of treatment. FACT‐An score showed a relevant improvement between t0 and t1 in these patients. At baseline, TCD showed a mean cerebral blood flow (CBF) velocity in the upper normal range. Abnormalities in brain function were observed in five patients. In the eight patients who were re‐evaluated at t1, improvement was observed in three responding patients, two of them with abnormal values at t0. A strict correlation between QOL and neurophysiological improvements was not observed.
Conclusions: A high‐dose induction phase with rHuEPO followed by maintenance therapy may be an effective therapeutic schedule for low‐risk MDS patients. The erythroid response was associated with positive changes in the QOL. Neurophysiological improvements occurred only in a part (50%) of responding patients, mainly those who showed altered results at baseline.
Millimetric continuum observations of the Galactic disk at b about -8 deg and l about 290-310 deg has been achieved during the Italian 1987-1988 scientific expedition in Antarctica. Emission was ...detected from few southern clouds (the Chamaeleon region and a cirrus cloud), and their energy distributions reveal an excess in the long wavelength range. This fact is interpreted as being due to the presence of a cold component of the interstellar material coexisting with the warm dust responsible for the emission detected by IRAS. Then, a multiple nature of the interstellar dust is suggested also in regions far from the Galactic bulge. 45 refs.
Millimetric observations (1-2 mm) toward the Magellanic Clouds from Antarctica show a good spatial correlation with the IRAS 100-micron emission in the same sky regions. Very likely a cold component ...coexisting with the warm dust detected by IRAS has been observed. 18 refs.
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