Haematologica
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The potential role of autologous stem cell transplantation (ASCT) as an alternative therapeutic strategy in chronic myelogenous leukemia (CML) has been widely explored in pilot studies, but the clinical results in terms of survival have so far been evaluated only retrospectively and in heterogeneous groups of patients. The goal of our prospective study was to evaluate the feasibility and long-term efficacy of unmanipulated ASCT followed by low-dose interferon-alpha in a homogeneous group of patients affected by CML in a very early phase of disease. ⋯ Our experience suggests that high-dose therapy followed by unmanipulated peripheral blood stem cell transplantation and low-dose interferon-alpha is a feasible approach, which results in long-term survival in newly diagnosed CML patients. These data need to be confirmed in controlled trials comparing ASCT with other therapeutic approaches, such as the use of interferon-alpha alone or in combination with other agents.
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Most cases of gastric low-grade mucosa-associated lymphoid tissue (MALT) lymphoma are associated with H. pylori. In localized disease (stage I), eradication of H. pylori can result in histologic regression of the lymphoma in 50% to 100% of the patients. Moreover, in half of the apparently cured patients a monoclonal rearrangement of the IgH gene can be demonstrated. However, data on the long-term outcome of the patients are scarce. We report the evolution of a series of patients followed-up since 1994 in order to evaluate the long-term outcome of the apparently cured lymphoma. ⋯ In most cases of gastric low-grade MALT lymphoma in stage I eradication of H. pylori can produce histologic regression of the lymphoma and this regression can be maintained for years. However, IgH gene monoclonality can be detected and persists in most cases. Although this persistent monoclonality seems to indicate the presence of a latent lymphoma population, over a period of 6 years it has not so far influenced the outcome. These findings indicate that in cases of localized gastric low-grade MALT lymphoma associated with H. pylori, the first step of treatment should be eradication of the H. pylori; however, a close and long follow-up is essential to determine the ultimate outcome of these patients and the possible significance of the persistent monoclonality.
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The risk of relapse after autologous bone marrow transplantation (ASCT) is high and is related to the type of malignancy and phase of the disease. The outcome for the patient who relapses after an autologous transplant is poor. Some of these patients achieve a remission with conventional chemotherapy, but it is usually short-lasting. Most of them succumb to the original disease. One further therapeutic possibility is an allogeneic transplant which would confer the potential advantage of a graft-versus-leukemia effect in addition to the lack of tumor contamination of the graft and to a high-dose intensity conditioning regimen. ⋯ These data suggest that patients relapsing after an autotransplant should be screened for potential related or unrelated donors: although TRM remains high there is a definite chance of long-term disease-free survival if these patients are allografted.
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Multicenter Study Clinical Trial
Treatment of childhood acute lymphoblastic leukemia. Long-term results of the AIEOP-ALL 87 study.
In March 1987 AIEOP started the AIEOP-ALL-87 study, based on the previous AIEOP-ALL-82. The aim of this new study was to evaluate, for all risk groups: a) the efficacy of treatment intensification achieved by adding a fourth drug (daunomycin) in the induction phase and a 3-drug reinduction phase for all risk groups; b) the impact of the addition of three doses of intrathecal methotrexate during cranial radiotherapy and extended exposure to weekly high-dose L-aspariginase during late intensification in high risk patients. We report the long-term results of the AIEOP ALL-87 study. ⋯ When compared to the results of the AIEOP-ALL-82 study, treatment intensification in the ALL-87 study has improved long-term survival and EFS from 66.4% and 53.6% to 74.7% and 62.8%, respectively. Failures were mostly due to marrow or extramedullary relapses suggesting that further treatment intensification, as being used in current therapeutic strategies, is appropriate, although patients relapsing after less intensive treatment may have better chances of rescue. These results, although obtained in a relatively large proportion of patients, in which infants were not included, indicate that the addition of high-dose L-asparaginase to a relatively non-intensive treatment may be of major benefit for HR patients and that the addition of intrathecal methotrexate during CRT, may improve the central nervous system-disease control with a marked reduction of nervous system relapses.
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The diagnostic criteria of the Polycythemia Vera Study Group (PVSG), although generally acknowledged as the gold standard for establishing a diagnosis of polycythemia vera (PV), do not consider bone marrow features. It may, therefore, be speculated that initial-early stages of PV are overlooked. In this retrospective study we tried to investigate whether bone marrow morphology of patients with an only borderline to slight increase in hemoglobin/hematocrit not conforming with the postulates of the PVSG enabled a clear-cut differentiation between PV and secondary (reactive) polycythemias (SP). ⋯ Initial-early PV is characterized by a specific pattern of bone marrow histopathology. Clinical features of distinctive impact include splenomegaly, thrombocyte count, LDH, LAP and in particular erythropoietin level. Taking these clinical and histologic findings into consideration, reactive-secondary causes of polycythemia (SP) are clearly distinguishable from autonomous ones (PV).