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1.
Eur J Haematol ; 101(1): 78-85, 2018 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-29569278

RESUMO

OBJECTIVE: The most typical cytogenetic aberration in myelodysplastic syndromes is del(5q), which, when isolated, is associated with refractory anaemia and good prognosis. Based on high rates of erythroid response and transfusion independence, Lenalidomide (LEN) became the standard treatment. This multi-centre study was designed to supplement Italian Registry data on LEN by addressing prescription, administration appropriateness, haematological and cytogenetic responses and disease evolution. METHODS: MORE study was an observational, non-interventional, multi-centre, retrospective and prospective study. Cases were recruited from 45 Haematological Centres throughout Italy. Data were collected from the Italian National Registry for Lenalidomide administration and supplemented by a MORE data form. RESULTS: Data from 190/213 patients were analysed. In all, 149 had been diagnosed by conventional cytogenetics (GROUP A) and 41 only by FISH (GROUP B). Overall erythroid response was obtained in 92.8% of cases. Overall cytogenetic remission was achieved in 22.6% of cases. Disease progression occurred in 15.6% of cases. Clonal cytogenetic evolution characterised progression to AML but not to higher risk MDS. CONCLUSIONS: Erythroid response to Lenalidomide was similar in MDS with isolated del(5q) and with del(5q) plus one anomaly. Progression to AML or higher risk MDS showed different cytogenetic features.


Assuntos
Deleção Cromossômica , Cromossomos Humanos Par 5/química , Fatores Imunológicos/uso terapêutico , Leucemia Mieloide Aguda/tratamento farmacológico , Síndromes Mielodisplásicas/tratamento farmacológico , Sistema de Registros , Talidomida/análogos & derivados , Idoso , Progressão da Doença , Feminino , Humanos , Itália , Cariotipagem , Lenalidomida , Leucemia Mieloide Aguda/etiologia , Leucemia Mieloide Aguda/genética , Leucemia Mieloide Aguda/imunologia , Masculino , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/complicações , Síndromes Mielodisplásicas/genética , Síndromes Mielodisplásicas/imunologia , Estudos Prospectivos , Indução de Remissão , Estudos Retrospectivos , Talidomida/uso terapêutico
2.
Eur J Haematol ; 97(6): 554-561, 2016 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-27124765

RESUMO

OBJECTIVES: In this paper, we present a review of critical concepts and research perspectives and produce recommendations on the optimal use of pixantrone in non-Hodgkin lymphoma (NHL) by group discussion from an expert panel appointed by the Italian Society of Hematology and the affiliate societies, Società Italiana di Ematologia Sperimentale and Gruppo Italiano Trapianto di Midollo Osseo. METHODS: Recommendations were produced using the Delphi process. Scientific evidence on pixantrone efficacy was analyzed using Grades of Recommendation, Assessment, Development, and Evaluation (GRADE) methodology in the areas where at least one randomized trial was published. The following key issues were addressed for practical recommendations: pixantrone monotherapy in aggressive relapsed or refractory non-Hodgkin B-cell lymphomas and toxicity risk management in patients candidates to pixantrone. RESULTS AND CONCLUSIONS: After a balanced and value-oriented discussion, the panel agreed that the benefit/risk profile was in favor of pixantrone in the treatment of adult patients with multiply relapsed or refractory aggressive NHL B-cell lymphomas. Pixantrone was deemed to be contraindicated in patients with uncontrolled cardiovascular disease. Despite a low rate of cardiotoxicity of pixantrone reported in clinical trials, the panel recommended that all patients receiving pixantrone should undergo periodical cardiac monitoring.


Assuntos
Antineoplásicos/uso terapêutico , Isoquinolinas/uso terapêutico , Linfoma não Hodgkin/tratamento farmacológico , Inibidores da Topoisomerase II/uso terapêutico , Antineoplásicos/farmacologia , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Terapia Combinada , Gerenciamento Clínico , Avaliação Pré-Clínica de Medicamentos , Resistencia a Medicamentos Antineoplásicos , Humanos , Isoquinolinas/farmacologia , Linfoma não Hodgkin/patologia , Guias de Prática Clínica como Assunto , Recidiva , Inibidores da Topoisomerase II/farmacologia
3.
Eur J Haematol ; 92(6): 527-36, 2014 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-24580147

RESUMO

BACKGROUND: In the absence of randomized, controlled trial data to support iron chelation therapy in transfusion-dependent patients with myelodysplastic syndromes (MDS), continued evidence from large prospective clinical trials evaluating the efficacy and safety of iron chelation therapy in this patient population is warranted. METHODS: The safety and efficacy of deferasirox was examined in a prospective, open-label, single-arm, multicenter trial of transfusion-dependent patients with International Prognostic Scoring System low- or intermediate-1-risk MDS and evidence of transfusion-related iron overload. The effects of deferasirox therapy on hematological response and disease progression were also examined. RESULTS: Of 159 participants enrolled from 37 Italian centers, 152 received ≥1 dose of deferasirox (initiated at 10-20 mg/kg/day and titrated as appropriate), and 68 completed the study. Of 84 patients who discontinued deferasirox therapy, 22 died during the trial, and 28 withdrew due to an adverse event (AE). Fourteen treatment-related grade 3 AEs occurred in 11 patients, whereas no grade 4 or 5 drug-related AEs were reported. Significant risks for dropout were a higher serum ferritin level at baseline, a higher MDS-Specific Comorbidity Index, and a shorter diagnosis-enrollment interval. Median serum ferritin level fell from 1966 ng/mL to 1475 ng/mL (P < 0.0001). The cumulative incidence of transfusion independence, adjusted for death and disease progression, was 2.6%, 12.3%, and 15.5% after 6, 9, and 12 months, respectively. CONCLUSIONS: Deferasirox therapy in transfusion-dependent patients with MDS was moderately well tolerated and effectively lowered serum ferritin levels. Positive hematological responses were observed, and a subset of patients achieved transfusion independence.


Assuntos
Benzoatos/uso terapêutico , Transfusão de Sangue , Quelantes de Ferro/uso terapêutico , Sobrecarga de Ferro/tratamento farmacológico , Sobrecarga de Ferro/etiologia , Síndromes Mielodisplásicas/complicações , Síndromes Mielodisplásicas/terapia , Triazóis/uso terapêutico , Adulto , Idoso , Idoso de 80 Anos ou mais , Benzoatos/efeitos adversos , Deferasirox , Feminino , Ferritinas/sangue , Humanos , Quelantes de Ferro/efeitos adversos , Masculino , Pessoa de Meia-Idade , Fatores de Risco , Reação Transfusional , Resultado do Tratamento , Triazóis/efeitos adversos , Adulto Jovem
4.
Haematologica ; 98(9): 1344-52, 2013 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-24006407

RESUMO

With the aim of reviewing critical concepts and producing recommendations for the management of chronic myelomonocytic leukemia, key questions were selected according to the criterion of clinical relevance. Recommendations were produced using a Delphi process and four consensus conferences involving a panel of experts appointed by the Italian Society of Hematology and affiliated societies. This report presents the final statements and recommendations, covering patient evaluation at diagnosis, diagnostic criteria, risk classification, first-line therapy, monitoring, second-line therapy and allogeneic stem cell transplantation. For the first-line therapy, the panel recommended that patients with myelodysplastic-type chronic myelomonocytic leukemia and less than 10% blasts in bone marrow should be managed with supportive therapy aimed at correcting cytopenias. In patients with myelodysplastic-type chronic myelomonocytic leukemia with a high number of blasts in bone marrow (≥ 10%), supportive therapy should be integrated with the use of 5-azacytidine. Patients with myeloproliferative-type chronic myelomonocytic leukemia with a low number of blasts (<10%) should be treated with cytoreductive therapy. Hydroxyurea is the drug of choice to control cell proliferation and to reduce organomegaly. Patients with myeloproliferative-type chronic myelomonocytic leukemia, and a high number of blasts should receive polychemotherapy. Both in myelodysplastic-type and myeloproliferative-type chronic myelomonocytic leukemia, allogeneic stem cell transplantation should be offered within clinical trials in selected patients.


Assuntos
Consenso , Leucemia Mielomonocítica Crônica/terapia , Guias de Prática Clínica como Assunto/normas , Sociedades Médicas/normas , Organização Mundial da Saúde , Gerenciamento Clínico , Humanos , Leucemia Mielomonocítica Crônica/diagnóstico
5.
Am J Hematol ; 88(3): 185-92, 2013 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-23339086

RESUMO

By using the GRADE system, we updated the guidelines for management of follicular cell lymphoma issued in 2006 from SIE, SIES, and GITMO group. We confirmed our recommendation to frontline chemoimmunotherapy in patients with Stage III-IV disease and/or high tumor burden. Maintenance rituximab was also recommended in responding patients. In patients relapsing after an interval longer than 12 months from frontline therapy, we recommended chemoimmunotherapy with non cross-resistant regimens followed by rituximab maintenance. High dose chemotherapy followed by hematopoietic stem cell transplant was recommended for young fit patients who achieve a response after salvage chemoimmunotherapy.


Assuntos
Anticorpos Monoclonais Murinos/uso terapêutico , Antineoplásicos/uso terapêutico , Transplante de Células-Tronco Hematopoéticas , Imunoterapia , Linfoma Folicular/terapia , Adulto , Anticorpos Monoclonais Murinos/farmacologia , Antineoplásicos/farmacologia , Gerenciamento Clínico , Esquema de Medicação , Feminino , Humanos , Linfoma Folicular/patologia , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Rituximab , Prevenção Secundária , Fatores de Tempo
6.
Ann Hematol ; 91(6): 875-88, 2012 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-22476884

RESUMO

In this project, we produced drug-specific recommendations targeting the use of new agents for multiple myeloma (MM). We used the GRADE (Grades of Recommendation, Assessment, Development, and Evaluation) system which separates the judgments on quality of evidence from the judgment about strength of recommendations. We recommended thalidomide and bortezomib in MM patients candidates to autologous stem cell transplantation (ASCT) (weak positive). We did not recommend novel agents as maintenance therapy after ASCT (weak negative). In patients not candidate to ASCT, thalidomide or bortezomib (strong positive) associated with melphalan and prednisone were recommended. In these patients, no specific course of action could be recommended as for maintenance therapy. In patients who are refractory or relapsing after first-line therapy, we recommended bortezomib and pegylated liposomal doxorubicin, or lenalidomide and dexamethasone combinations (weak positive).


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Prática Clínica Baseada em Evidências , Hematologia/métodos , Mieloma Múltiplo/tratamento farmacológico , Guias de Prática Clínica como Assunto , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Ácidos Borônicos/administração & dosagem , Bortezomib , Hematologia/legislação & jurisprudência , Hematologia/organização & administração , Humanos , Lenalidomida , Mieloma Múltiplo/patologia , Gradação de Tumores/métodos , Pirazinas/administração & dosagem , Sociedades Médicas/legislação & jurisprudência , Sociedades Médicas/organização & administração , Talidomida/administração & dosagem , Talidomida/análogos & derivados
7.
Ann Hematol ; 91(4): 527-32, 2012 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-21964878

RESUMO

Essential thrombocythaemia (ET) is a myeloproliferative neoplasm characterized by elevated platelet counts and increased incidence of thrombosis and haemorrhage. Median age at diagnosis is 65-70 years. Life expectancy is similar to that of the healthy population. Symptoms and complications may affect quality of life (QoL); in particular, in elderly patients ET may represent an additional burden. We performed a survey in 494 elderly ET patients to evaluate patient-reported outcomes (PROs). Comorbidities were present in 305 (62%) patients. Factorial analysis based on survey items representing psychological aspects of daily life identified an "attitude domain" with four clusters of patients: (A) very pessimistic (n = 99), (B) pessimistic (n = 101), (C) optimistic (n = 90), and (D) very optimistic (n = 107). Patients in cluster A had more comorbidities (p = 0.003) while patients in cluster D required fewer medical visits and were less disturbed by medications (p < 0.0001). Independent factors predicting Short-Form Health Survey, version 2 physical QoL were grade of optimism (p < 0.0001), gender (p = 0.007), and Charlson comorbidity index (p < 0.0001)). Grade of optimism and disturbances related to medication predicted mental QOL (p < 0.0001). In conclusion, physicians should take into consideration PROs, as "attitude" is associated with physical and mental QoL. Treatment should be tailored to patients' needs according to comorbidities, lifestyle, and psychological conditions.


Assuntos
Qualidade de Vida , Trombocitemia Essencial/fisiopatologia , Idoso , Estudos Transversais , Feminino , Humanos , Itália , Masculino , Pessoa de Meia-Idade , Inquéritos e Questionários , Trombocitemia Essencial/patologia
8.
Haematologica ; 94(1): 102-12, 2009 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-19001282

RESUMO

As many options are now available to treat patients with de novo acute myeloid leukemia, the Italian Society of Hematology and two affiliated societies (SIES and GITMO) commissioned project to an Expert Panel aimed at developing clinical practice guidelines for acute myeloid leukemia treatment. After systematic comprehensive literature review, the Expert Panel formulated recommendations for the management of primary acute myeloid leukemia (with the exception of acute promyelocytic leukemia) and graded them according to the supporting evidence. When evidence was lacking, consensus-based statements have been added. First-line therapy for all newly diagnosed patients eligible for intensive treatment should include one cycle of induction with standard dose cytarabine and an anthracycline. After achieving complete remission, patients aged less than 60 years should receive consolidation therapy including high-dose cytarabine. Myeloablative allogeneic stem cell transplantation from an HLA-compatible sibling should be performed in first complete remission: 1) in children with intermediate-high risk cytogenetics or who achieved first complete remission after the second course of therapy; 2) in adults less than 40 years with an intermediate-risk; in those aged less than 55 years with either high-risk cytogenetics or who achieved first complete remission after the second course of therapy. Stem cell transplantation from an unrelated donor is recommended to be performed in first complete remission in adults 30 years old or younger, and in children with very high-risk disease lacking a sibling donor. Alternative donor stem cell transplantation is an option in high-risk patients without a matched donor who urgently need transplantation. Patients aged less than 60 years, who either are not candidate for allogeneic stem cell transplantation or lack a donor, are candidates for autologous stem cell transplantation. We describe the results of a systematic literature review and an explicit approach to consensus techniques, which resulted in recommendations for the management of primary non-APL acute myeloid leukemia.


Assuntos
Transplante de Medula Óssea , Leucemia Mieloide/terapia , Guias de Prática Clínica como Assunto/normas , Hematologia , Humanos , Itália/epidemiologia , Leucemia Mieloide/epidemiologia , Leucemia Mieloide/genética , Leucemia Mieloide/cirurgia , Sociedades Médicas
9.
Haematologica ; 94(4): 550-65, 2009 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-19278966

RESUMO

The Italian Society of Hematology (SIE), the Italian Society of Experimental Haematology (SIES) and the Italian Group for Bone Marrow Transplantation (GITMO) commissioned a project to develop practice guidelines for the initial work-up, therapy and follow-up of classical Hodgkin's lymphoma. Key questions to the clinical evaluation and treatment of this disease were formulated by an Advisory Committee, discussed and approved by an Expert Panel (EP) composed of senior hematologists and one radiotherapist. After a comprehensive and systematic literature review, the EP recommendations were graded according to their supporting evidence. An explicit approach to consensus methodologies was used for evidence interpretation and for producing recommendations in the absence of a strong evidence. The EP decided that the target domain of the guidelines should include only classical Hodgkin's lymphoma, as defined by the WHO classification, and exclude lymphocyte predominant histology. Distinct recommendations were produced for initial work-up, first-line therapy of early and advanced stage disease, monitoring procedures and salvage therapy, including hemopoietic stem cell transplant. Separate recommendations were formulated for elderly patients. Pre-treatment volumetric CT scan of the neck, thorax, abdomen, and pelvis is mandatory, while FDG-PET is recommended. As to the therapy of early stage disease, a combined modality approach is still recommended with ABVD followed by involved-field radiotherapy; the number of courses of ABVD will depend on the patient risk category (favorable or unfavorable). Full-term chemotherapy with ABVD is recommended in advanced stage disease; adjuvant radiotherapy in patients without initial bulk who achieved a complete remission is not recommended. In the elderly, chemotherapy regimens more intensive than ABVD are not recommended. Early evaluation of response with FDG-PET scan is suggested. Relapsed or refractory patients should receive high-dose chemotherapy and autologous hemopoietic stem cells transplant. Allogeneic transplant is recommended in patients relapsing after autologous transplant. All fertile patients should be informed of the possible effects of therapy on gonadal function and fertility preservation measures should be taken before the initiation of therapy.


Assuntos
Transplante de Medula Óssea , Doença de Hodgkin/terapia , Classificação , Doença de Hodgkin/classificação , Doença de Hodgkin/diagnóstico , Humanos , Itália , Tomografia por Emissão de Pósitrons/métodos , Terapia de Salvação/métodos , Sociedades Médicas , Organização Mundial da Saúde
10.
Leuk Res ; 81: 88-94, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-31055248

RESUMO

The introduction of new therapeutic agents in chronic lymphocytic leukemia (CLL), including the new kinase inhibitors (KIs) ibrutinib and idelalisib, has changed the therapeutic landscape of the disease. The new KIs have also changed frequency and epidemiology of infections, that represent a major cause of morbidity and mortality of the disease. Hence, the great strides in the indications and use of new KIs need parallel amelioration of prophylaxis and supportive treatment for infections. Moving from the recognition that infection control represents an unmet need, the Italian Society of Hematology (SIE) convened a panel of experts who had published and/or expressed an interest in infection complications in CLL. The goal of the project was to provide practice recommendations for the management of the infectious complications of CLL during ibrutinib or idelalisib therapy. The present publication represents the results of a series of email correspondences and meetings held during 2017 and 2018. Three domains of infectious complications during KIs therapy for CLL were explored: risk assessment, risk management and risk monitoring. We hope these recommendations will help to minimize infectious adverse events, and we believe that an optimal management of them will be rewarded by better outcomes, and better quality of life.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Controle de Infecções/normas , Infecções/terapia , Leucemia Linfocítica Crônica de Células B/tratamento farmacológico , Guias de Prática Clínica como Assunto/normas , Qualidade de Vida , Adenina/análogos & derivados , Hematologia , Humanos , Infecções/induzido quimicamente , Infecções/patologia , Itália , Piperidinas , Purinas/administração & dosagem , Pirazóis/administração & dosagem , Pirimidinas/administração & dosagem , Quinazolinonas/administração & dosagem
11.
Haematologica ; 93(5): 741-52, 2008 May.
Artigo em Inglês | MEDLINE | ID: mdl-18413891

RESUMO

New measures of iron accumulation in liver and heart (superconducting quantum inference device and magnetic resonance imaging), and oral iron chelators (deferiprone and deferasirox) are available for managing iron overload in thalassemia major. To assure appropriate use of these new health technologies, the Italian Society of Hematology appointed a panel of experts to produce clinical practice-guidelines for the management of iron overload in thalassemia major and related disorders. The analytical hierarchy process, a technique for multicriteria decision analysis, was applied to relevant key questions in order to identify the alternative strategies, generate explicit criteria for their evaluation, and check how well the alternatives fulfilled the criteria. The result of a comprehensive systematic review of articles released from 1990 to 2007 was used as a source of scientific evidence to compare the decisional options pairwise, and select the final recommendation. Every step in the model was developed from questionnaires and group discussion. The resulting recommendations advise about which examination to carry out in order to plan iron chelation therapy, when to start iron chelation, which iron chelator to choose in regularly transfused patients, how to monitor iron chelation therapy, and when and how to switch standard therapy.


Assuntos
Hematologia/métodos , Sobrecarga de Ferro/terapia , Guias de Prática Clínica como Assunto , Talassemia beta/terapia , Adolescente , Adulto , Benzoatos/uso terapêutico , Criança , Técnicas de Apoio para a Decisão , Deferasirox , Deferiprona , Desferroxamina/uso terapêutico , Hematologia/tendências , Humanos , Quelantes de Ferro/uso terapêutico , Fígado/metabolismo , Piridonas/uso terapêutico , Sideróforos/uso terapêutico , Triazóis/uso terapêutico
12.
Haematologica ; 93(9): 1364-71, 2008 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-18603558

RESUMO

Extranodal non-Hodgkin's lymphomas constitute 20-25% of overall non-Hodgkin's lymphomas cases and can be managed with very different therapeutic strategies. Therefore, the Italian Society of Hematology and the two affiliate societies (the Italian Society of Experimental Hematology and the Italian Group of Bone Marrow Transplantation) appointed a panel of experts to produce clinical practice-guidelines for the management of these conditions. Primary lung and mediastinal lymphomas were the objective of this part of the project. The panel of experts produced the following key recommendations that were graded according to the strength of evidence and clinical judgement. The first-line therapy for non-MALT primary lung non-Hodgkin's lymphomas should include anthracycline-based chemotherapy with CHOP or CHOP-like, MACOP-B or MACOP-B-like regimens (grade D). Rituximab association with chemotherapy needs to be evaluated within approved clinical trials. Second-line therapy with high-dose chemotherapy and autologous stem cell transplantation is recommended (grade B). In patients with MALT primary lung non-Hodgkin's lymphomas, the recommended first-line therapy should include chlorambucil, CHOP, CHOP-like or fludarabine-containing regimens (grade B). Radiotherapy is to be reserved for patients with a unique, small lesion in a poorly mobile site and with contraindication to surgery (grade D). Rituximab should be administered only within approved clinical trials. For treatment of primary mediastinal large B-cell lymphomas, the recommended first-line therapy is a chemotherapy and radiotherapy association (grade B). An anthracycline-based chemotherapy with CHOP, MACOP-B or VACOP-B is recommended (grade B). Rituximab combination with chemotherapy is highly suggested but only for patients enrolled into approved clinical trials. Patients with an inadequate early response should be candidates for early intensification with high-dose chemotherapy (grade C). Patients with refractory or relapsed disease should undergo rescue programs including intensive, non-cross-resistant debulking treatment followed, in chemosensitive patients, by high-dose chemotherapy and autologous stem cell transplantation (grade B).


Assuntos
Transplante de Medula Óssea , Neoplasias Pulmonares/terapia , Linfoma não Hodgkin/terapia , Neoplasias do Mediastino/terapia , Guias de Prática Clínica como Assunto/normas , Adulto , Terapia Combinada , Feminino , Hematologia , Humanos , Síndromes de Imunodeficiência , Itália , Neoplasias Pulmonares/patologia , Linfoma não Hodgkin/classificação , Linfoma não Hodgkin/patologia , Masculino , Neoplasias do Mediastino/patologia , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Recidiva , Sociedades Médicas
13.
Haematologica ; 93(12): 1877-85, 2008 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-18838473

RESUMO

Tumor lysis syndrome is a potentially life threatening complication of massive cellular lysis in cancers. Identification of high-risk patients and early recognition of the syndrome is crucial in the institution of appropriate treatments. Drugs that act on the metabolic pathway of uric acid to allantoin, like allopurinol or rasburicase, are effective for prophylaxis and treatment of tumor lysis syndrome. Sound recommendations should regulate diagnosis and drug application in the clinical setting. The current article reports the recommendations on the management of tumor lysis syndrome that were issued during a Consensus Conference project, and which were endorsed by the Italian Society of Hematology (SIE), the Italian Association of Pediatric Oncologists (AIEOP) and the Italian Society of Medical Oncology (AIOM). Current concepts on the pathophysiology, clinical features, and therapy of tumor lysis syndrome were evaluated by a Panel of 8 experts. A consensus was then developed for statements regarding key questions on tumor lysis syndrome management selected according to the criterion of relevance by group discussion. Hydration and rasburicase should be administered to adult cancer patients who are candidates for tumor-specific therapy and who carry a high risk of tumor lysis syndrome. Cancer patients with a low-risk of tumor lysis syndrome should instead receive hydration along with oral allopurinol. Hydration and rasburicase should also be administered to patients with clinical tumor lysis syndrome and to adults and high-risk children who develop laboratory tumor lysis syndrome. In conclusion, the Panel recommended rasburicase for tumor lysis syndrome prophylaxis in selected patients based on the drug efficacy profile. Methodologically rigorous studies are needed to clarify its cost-effectiveness profile.


Assuntos
Síndrome de Lise Tumoral/tratamento farmacológico , Alopurinol/uso terapêutico , Gerenciamento Clínico , Neoplasias Hematológicas/complicações , Humanos , Itália , Urato Oxidase/uso terapêutico
14.
Eur J Haematol ; 80(1): 31-6, 2008 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-18036184

RESUMO

T(4;14) chromosomal abnormality is one of the most adverse prognostic factors predicting for poor outcome in multiple myeloma (MM) patients. It has been recently suggested that bone disease, as evaluated by spinal magnetic resonance imaging (MRI), is relatively infrequent in these patients. In the present study, we aimed at further testing this hypothesis by analyzing the extent of whole bone involvement in patients showing t(4;14) chromosomal translocation as compared with negative patients. For this purpose, 53 consecutive newly diagnosed MM patients (35M, 18F, median age = 55 yr) underwent evaluation of total skeletal X-ray, whole spine MRI, and at the same time, quantification of markers of bone resorption (urinary N-terminal telopeptide, pyridinoline, deoxypyridinoline, serum crosslaps), and bone formation (bone alkaline phosphatase and osteocalcin) was performed. The presence of IgH/MMSET fusion gene as a surrogate marker for t(4;14), was detected in 11 patients (20.7%), whose clinical characteristics were similar to those observed in t(4;14) negative patients. The type of marrow involvement at spinal MRI (diffuse vs. focal vs. negative) was the same in both groups of patients, even though overt vertebral fractures were more frequently found in t(4;14) positive cases (82% vs. 43%, P = 0.05); in line with this finding, skeletal lesions were more common in t(4;14) positive patients (mean skeletal score = 8.54 +/- 1.36 SE, as compared with 3.42 +/- 0.57 SE in t(4;14) negative cases, P = 0.000). These data were confirmed by the evaluation of serum crosslaps, that were significantly increased in patients with t(4;14) abnormality as compared with negative individuals (10,400 pmol/L +/- 2160 SE vs. 5640 pmol/L +/- 859 SE P = 0.02) Our results indicate that, at variance to what has been previously reported, bone resorption is more prominent in t(4;14) positive patients.


Assuntos
Doenças Ósseas/diagnóstico , Reabsorção Óssea/diagnóstico , Mieloma Múltiplo/complicações , Mieloma Múltiplo/genética , Translocação Genética , Adulto , Idoso , Doenças Ósseas/etiologia , Reabsorção Óssea/etiologia , Cromossomos Humanos Par 14 , Cromossomos Humanos Par 4 , Feminino , Histona-Lisina N-Metiltransferase/genética , Humanos , Masculino , Pessoa de Meia-Idade , Receptor Tipo 3 de Fator de Crescimento de Fibroblastos/genética , Proteínas Repressoras/genética
15.
Exp Hematol ; 34(7): 879-87, 2006 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-16797415

RESUMO

OBJECTIVE: Altered self-antigen processing/presentation of apoptotic cells by DCs and/or modifications of autoantigens may lead to the development of autoantibodies. Increasing evidence indicates that platelets may undergo apoptosis. Therefore, in the present study we investigated whether platelet apoptosis and/or dendritic cells (DCs) may play a role in the stimulation of the immuno-mediated anti-platelet response in chronic immune thrombocytopenic purpura (ITP). METHODS AND RESULTS: Twenty-nine patients with active ITP and 29 healthy adult volunteers were enrolled into the study. Freshly washed platelets and platelets aged in a plasma-free buffer for 72 hours at 37 degrees C were assessed by flow cytometry for phosphatidylserine exposure using annexin V-FITC, caspase activation, and platelet activation markers. CD14-derived DCs were characterized by immunophenotyping, cytokine production, and ability to present fresh and aged platelets to T lymphocytes. We demonstrated that platelets from ITP patients, either fresh or in vitro aged, show increased apoptosis (with low levels of activation) in comparison to their normal counterparts. We also found that immature DCs readily ingest apoptotic platelets. Furthermore, in ITP patients DCs, prepulsed with autologous/allogeneic fresh and aged platelets, are highly efficient in stimulating autologous T-cell proliferation as compared to DCs derived from healthy donors. This finding may be related to the upregulated expression of CD86 in DCs from ITP patients and not to higher phagocytic activity. CONCLUSION: These results suggest that DC dysfunction, together with increased propensity of platelets to undergo apoptosis, may play a role in the stimulation of the immune system in ITP.


Assuntos
Apoptose , Plaquetas/imunologia , Células Dendríticas/imunologia , Púrpura Trombocitopênica Idiopática/imunologia , Linfócitos T/imunologia , Adulto , Idoso , Feminino , Citometria de Fluxo , Humanos , Imunofenotipagem , Masculino , Pessoa de Meia-Idade
16.
Leuk Res ; 62: 108-115, 2017 11.
Artigo em Inglês | MEDLINE | ID: mdl-29054020

RESUMO

Myelodysplastic syndromes (MDS) are a group of clonally-acquired blood disorders characterized by ineffective hematopoiesis leading to cytopenias. Red blood cell transfusions are an important component of supportive care in patients with MDS. Prolonged exposure to transfusions can lead to iron overload, which results in iron-induced toxicity caused by the production of reactive oxygen species (ROS). ROS accumulation has detrimental effects also on hematopoietic stem cells and may contribute to MDS progression. The observation that iron chelation improves hematologic parameters and reduces transfusion dependence further indicates that iron overload impairs hematopoiesis. Over the past decade, the mechanisms regulating iron homeostasis and the complex interplay between iron overload and toxicity, ineffective hematopoiesis, and transformation to leukemia have become clearer. In this narrative review, we provide an overview of recent findings pertaining to iron overload in patients with MDS and its effects on hematopoiesis. We also briefly discuss the position of chelation therapy in the context of the new developments.


Assuntos
Hematopoese/fisiologia , Sobrecarga de Ferro/etiologia , Síndromes Mielodisplásicas/complicações , Animais , Transfusão de Eritrócitos/efeitos adversos , Humanos , Sobrecarga de Ferro/prevenção & controle , Síndromes Mielodisplásicas/terapia
17.
Leuk Res ; 58: 63-72, 2017 07.
Artigo em Inglês | MEDLINE | ID: mdl-28460339

RESUMO

The discovery that Philadelphia-negative classical myeloproliferative neoplasms (MPNs) present with several molecular abnormalities, including the mostly represented JAK2V617F mutation, opened new horizons in the diagnosis, prognosis, and monitoring of these disorders. However, the great strides in the knowledge on molecular genetics need parallel progresses on the best approach to methods for detecting and reporting disease-associated mutations, and to shape the most effective and rationale testing pathway in the diagnosis, prognosis and monitoring of MPNs. The MPN taskforce of the Italian Society of Hematology (SIE) assessed the scientific literature and composed a framework of the best, possibly evidence-based, recommendations for optimal molecular methods as well as insights about the applicability and interpretation of those tests in the clinical practice, and clinical decision for testing MPNs patients. The issues dealt with: source of samples and nucleic acid template, the most appropriate molecular abnormalities and related detection methods required for diagnosis, prognosis, and monitoring of MPNs, how to report a diagnostic molecular test, calibration and quality control. For each of these issues, practice recommendations were provided.


Assuntos
Técnicas de Diagnóstico Molecular , Transtornos Mieloproliferativos/diagnóstico , Transtornos Mieloproliferativos/genética , Humanos , Itália
19.
Haematologica ; 91(1): 96-103, 2006 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-16434377

RESUMO

The Italian Society of Hematology (SIE) and two affiliate societies (SIES and GITMO) commissioned a project to develop clinical practice guidelines for the treatment of nodal diffuse large B-cell non Hodgkin lymphomas (DLBCL). Key questions clinically relevant to the management of patients with nodal DLBCL were formulated by an Advisory Committee (AC) and approved by an Expert Panel (EP) composed of eight senior hematologists. After a comprehensive and systematic literature review, the EP formulated therapy recommendations and graded them according to the supporting evidence. An explicit approach to consensus methodologies was used for evidence interpretation and for producing recommendations in the absence of strong evidence. The EP formulated recommendations on which first-line therapy to choose in patients with nodal DLBCL. Patients of all ages, with stage I-II disease and no adverse prognostic factors should receive abbreviated chemotherapy with an anthracycline-containing regimen plus involved field radiotherapy (35-40 Gy). Patients with stage I-II disease and at least one adverse prognostic factor, or with stage III-IV disease, should receive frontline chemoimmunotherapy with CHOP, CHOP-like or third-generation chemotherapy plus rituximab. Recommendations on stem cell transplantation and on which therapy to adopt for refractory or relapsed patients were also formulated.


Assuntos
Linfoma Difuso de Grandes Células B/terapia , Antineoplásicos/uso terapêutico , Gerenciamento Clínico , Transplante de Células-Tronco Hematopoéticas , Humanos , Itália , Linfonodos/patologia , Linfoma de Células B/terapia
20.
Haematologica ; 91(12): 1662-73, 2006 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-17145603

RESUMO

OBJECTIVES: The Italian Society of Hematology (SIE) and two affiliate societies (SIES and GITMO) commissioned a project to develop clinical practice guidelines for the treatment of chronic lymphocytic leukemia (CLL). METHODS: Key questions in the management of patients with CLL were formulated by an Advisory Committee and approved by an Expert Panel of eight senior hematologists. After a systematic review of the literature, recommendations for disease-specific and supportive therapies were formulated and graded according to the supporting evidence. Explicit consensus methods were used for providing recommendations for questions with incomplete or potentially biased evidence. RESULTS: It is recommended that therapy is commenced in patients with CLL when at least one of the following are present: B-symptoms, progressive/obstructive lymphadenopathy or organomegaly, rapid lymphocyte doubling time, anemia or thrombocytopenia (of new onset, worsening or steroid-resistant). It is recommended that patients without co-morbidity should receive fludarabine plus cyclophosphamide, whereas elderly patients with co-morbidity should receive oral chlorambucil. Younger patients with unfavorable biological risk factors should be considered for high-dose chemotherapy and autologous or allogeneic stem cell transplantation within approved clinical trials. Patients either relapsing rapidly after, or non-responsive to, first-line chlorambucil should receive fludarabine-containing regimens. Patients either relapsing soon after or not responding to fludarabine-based chemotherapy should be considered for schedules including non-cross-reactive agents, such as alemtuzumab, possibly followed by high-dose chemotherapy and autologous transplantation in the context of a clinical trial or by allogeneic stem cell transplantation. CONCLUSIONS: We describe the results of a systematic literature review and an explicit approach to consensus techniques which resulted in recommendations for the key therapeutic decisions in patients with CLL.


Assuntos
Transplante de Medula Óssea/normas , Hematologia/normas , Leucemia Linfocítica Crônica de Células B/terapia , Sociedades Médicas/normas , Gerenciamento Clínico , Humanos , Itália , Leucemia Linfocítica Crônica de Células B/diagnóstico
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