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1.
Clin Lymphoma Myeloma Leuk ; 20(8): e461-e467, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32507387

RESUMO

INTRODUCTION: Von Willebrand factor (vWF) cleaving protease ADAMTS-13 has a key role for maintaining normal size of vWF. A deficiency or dysfunction of vWF cleaving protease is associated with ultra large vWF multimers and thrombotic microangiopathy. Patients with cancers have reduced levels of vWF cleaving protease. In this pilot study, we have evaluated whether or not deficiencies of ADAMTS-13 were present in myelodysplastic syndromes (MDS). Moreover, we assessed if a reduction in basal levels of ADAMTS-13 may play a role in the prognosis of MDS. PATIENTS AND METHODS: We measured and compared the levels of vWF cleaving protease ADAMTS-13 in 100 patients with MDS and 35 healthy controls. Patients were divided into 2 groups according to the International Prognostic Scoring System: group I consisting of 44 patients with low-risk MDS and group II of 56 patients with high-risk MDS. Patients with high-risk and low-risk MDS presented significantly lower levels of ADAMTS-13 than controls (P < .001 and P = .0177, respectively). High-risk patients had significantly lower levels of ADAMTS-13 when compared with the low-risk group (P < .001). RESULTS: We found that reduced levels of ADAMTS-13 have a relationship with overall survival (P < .001). Statistical analysis showed that ADAMTS-13 correlates with cytogenetics (P < .001) and a tendency of slight correlation with platelet count and basal levels of ADAMTS-13 (R, 0.35; P value, 0.001). Moreover, we found that levels of ADAMTS-13 have correlation with response to treatment (P < .001). CONCLUSIONS: ADAMTS-13 in MDS might represent a surrogate marker of prognosis, response to therapy, or disease progression. Further studies are needed.

3.
Artigo em Inglês | MEDLINE | ID: mdl-32294049

RESUMO

HIV-positive patients have a 60- to 200-fold increased incidence of non-Hodgkin lymphomas (NHL) because of their impaired cellular immunity. Some NHL are considered acquired immunodeficiency syndrome (AIDS) defining conditions. Diffuse large B-cell Lymphoma (DLBC) and Burkitt lymphoma (BL) are the most commonly observed, whereas primary effusion lymphoma (PEL), Central Nervous System Lymphomas (PCNSL), plasmablastic lymphoma (PBL) and classic Hodgkin lymphoma (HL) are far less frequent. Multicentric Castleman disease (MCD) is an aggressive lymphoproliferative disorder highly prevalent in HIV-positive patients and strongly associated with HHV-8 virus infection. In the preCombination Antiretroviral Therapy (CART) era, patients with HIV-associated lymphoma had poor outcomes with median survivals of 5 to 6 months. By improving the immunological status, CARTs extended the therapeutic options for HIV positive patients with lymphomas, allowing them to tolerate standard chemotherapies regimen with similar outcomes to those of general population. The combination of CARTs and chemotherapy/immuno-chemotherapy treatment has resulted in a remarkable prolongation of survival among HIV-infected patients with lymphomas. In this short communication we briefly review the problems linked with the treatment of lymphoproliferative diseases in the HIV patients. Combination antiretroviral therapy (CARTs) not only reduces HIV replication and restores the immunological status improving immune function of the HIV-related lymphomas patients but allows patients to deal with standard doses of chemotherapies. The association of CARTs and chemotherapy allowed to obtain better results in terms of overall survival and complete responses. In the setting of HIV-associated lymphomas many issues remain open and their treatment is complicated by the patient's immunocompromised status and the need to treat HIV concurrently.

5.
J Clin Oncol ; 37(15): 1336-1351, 2019 05 20.
Artigo em Inglês | MEDLINE | ID: mdl-30969847

RESUMO

PURPOSE: To update the American Society of Clinical Oncology (ASCO)/American Society of Hematology (ASH) recommendations for use of erythropoiesis-stimulating agents (ESAs) in patients with cancer. METHODS: PubMed and the Cochrane Library were searched for randomized controlled trials (RCTs) and meta-analyses of RCTs in patients with cancer published from January 31, 2010, through May 14, 2018. For biosimilar ESAs, the literature search was expanded to include meta-analyses and RCTs in patients with cancer or chronic kidney disease and cohort studies in patients with cancer due to limited RCT evidence in the cancer setting. ASCO and ASH convened an Expert Panel to review the evidence and revise previous recommendations as needed. RESULTS: The primary literature review included 15 meta-analyses of RCTs and two RCTs. A growing body of evidence suggests that adding iron to treatment with an ESA may improve hematopoietic response and reduce the likelihood of RBC transfusion. The biosimilar literature review suggested that biosimilars of epoetin alfa have similar efficacy and safety to reference products, although evidence in cancer remains limited. RECOMMENDATIONS: ESAs (including biosimilars) may be offered to patients with chemotherapy-associated anemia whose cancer treatment is not curative in intent and whose hemoglobin has declined to < 10 g/dL. RBC transfusion is also an option. With the exception of selected patients with myelodysplastic syndromes, ESAs should not be offered to most patients with nonchemotherapy-associated anemia. During ESA treatment, hemoglobin may be increased to the lowest concentration needed to avoid transfusions. Iron replacement may be used to improve hemoglobin response and reduce RBC transfusions for patients receiving ESA with or without iron deficiency. Additional information is available at www.asco.org/supportive-care-guidelines and www.hematology.org/guidelines .


Assuntos
Anemia/tratamento farmacológico , Hematínicos/administração & dosagem , Neoplasias/sangue , Anemia/sangue , Medicamentos Biossimilares/administração & dosagem , Humanos , Metanálise como Assunto , Ensaios Clínicos Controlados Aleatórios como Assunto
6.
Blood Adv ; 3(8): 1197-1210, 2019 04 23.
Artigo em Inglês | MEDLINE | ID: mdl-30971397

RESUMO

PURPOSE: To update the American Society of Clinical Oncology (ASCO)/American Society of Hematology (ASH) recommendations for use of erythropoiesis-stimulating agents (ESAs) in patients with cancer. METHODS: PubMed and the Cochrane Library were searched for randomized controlled trials (RCTs) and meta-analyses of RCTs in patients with cancer published from January 31, 2010, through May 14, 2018. For biosimilar ESAs, the literature search was expanded to include meta-analyses and RCTs in patients with cancer or chronic kidney disease and cohort studies in patients with cancer due to limited RCT evidence in the cancer setting. ASCO and ASH convened an Expert Panel to review the evidence and revise previous recommendations as needed. RESULTS: The primary literature review included 15 meta-analyses of RCTs and two RCTs. A growing body of evidence suggests that adding iron to treatment with an ESA may improve hematopoietic response and reduce the likelihood of RBC transfusion. The biosimilar literature review suggested that biosimilars of epoetin alfa have similar efficacy and safety to reference products, although evidence in cancer remains limited. RECOMMENDATIONS: ESAs (including biosimilars) may be offered to patients with chemotherapy-associated anemia whose cancer treatment is not curative in intent and whose hemoglobin has declined to < 10 g/dL. RBC transfusion is also an option. With the exception of selected patients with myelodysplastic syndromes, ESAs should not be offered to most patients with nonchemotherapy-associated anemia. During ESA treatment, hemoglobin may be increased to the lowest concentration needed to avoid transfusions. Iron replacement may be used to improve hemoglobin response and reduce RBC transfusions for patients receiving ESA with or without iron deficiency. Additional information is available at www.asco.org/supportive-care-guidelines and www.hematology.org/guidelines.


Assuntos
Anemia/tratamento farmacológico , Medicamentos Biossimilares/uso terapêutico , Hematínicos/uso terapêutico , Neoplasias/tratamento farmacológico , Anemia/etiologia , Anemia/metabolismo , Anemia/patologia , Hematologia , Humanos , Neoplasias/metabolismo , Neoplasias/patologia , Guias de Prática Clínica como Assunto , Sociedades Médicas , Estados Unidos
8.
Med Oncol ; 35(9): 118, 2018 Aug 02.
Artigo em Inglês | MEDLINE | ID: mdl-30073422

RESUMO

Angioedema due to acquired deficiency of the inhibitor of the first component of complement (C1-INH) is a rare disease known as acquired angioedema (AAE). About 70% of patients with AEE display autoantibodies to C1-INH, the remaining patients have no antibodies to C1-INH. The clinical features of C1-INH deficiency include recurrent, self-limiting local swellings involving the skin, the gastrointestinal tract, and the upper respiratory tract. Swelling is due to accumulation of bradykinin released from high molecular weight kininogen. Patients with angioedema due to acquired C1 inhibitor deficiency (AEE) often have an associated lymphoproliferative disease including Non-Hodgkin Lymphomas (NHL). Among AAE patients with NHL, splenic marginal zone lymphoma (SMZL) has a higher prevalence (66%) compared to general population (2%) In the present study, we focused on patients with SMZL in AAE. We found 24 AAE patients with NHL and, among them 15 SMZL (62.5% of all NHL). We found NOTCH 2 activation in 4 /15 patients (26.6%) with SMZL, while no patients carried MYD 88 or BIRC3 mutations. Restricted immunoglobulin gene repertoire analysis showed that the IGHV1-2*04 allele was found to be over-represented in the group of patients with or without lymphoproliferative disease presenting with autoantibodies to C1-INH (41 of 55 (75%) of patients; p value 0.011) when compared to the control group of patients with AEE without antibodies to C1-INH, (7 of 27 (26%) of patients). Immunophenotyping failed to demonstrate the presence of autoreactive clones against C1-inhibitor. Taken together, these findings suggest a role for antigenic stimulation in the pathogenesis of lymphomas associated with AEE.


Assuntos
Angioedema Hereditário Tipos I e II/complicações , Linfoma de Zona Marginal Tipo Células B/etiologia , Neoplasias Esplênicas/etiologia , Idoso , Idoso de 80 Anos ou mais , Proteína Inibidora do Complemento C1 , Feminino , Humanos , Linfoma de Zona Marginal Tipo Células B/epidemiologia , Masculino , Pessoa de Meia-Idade , Neoplasias Esplênicas/epidemiologia
10.
Med Oncol ; 35(5): 76, 2018 Apr 19.
Artigo em Inglês | MEDLINE | ID: mdl-29675620

RESUMO

The myelodysplastic syndromes (MDSs) are clonal hematopoietic stem cell disorders. The International Prognostic Score System (IPSS) groups MDS in lower-risk (IPSS low and intermediate-1) and higher-risk disease (IPSS intermediate-2 and high). AML transformation is the main concern in higher-risk MDS, while anemia and transfusion dependency represent the major issues for low-risk MDS patients. Improving erythropoiesis, and eliminating fatigue and symptoms, is the main therapeutic goal for low-risk MDS patients. Around 50% of MDS patients present with anemia with an Hb level < 100 g/L. Severe anemia increases the negative effects of comorbidities, such as heart and lung failure. Erythropoiesis-stimulating agents (ESAs), with or without granulocyte colony-stimulating factor, induce erythroid response rates in 40-50% of lower-risk anemic MDS patients. The median response duration of 24 months. Apoptosis of erythroid cells is inhibited by ESAs leading to erythrocyte production. Our paper considers the state of the art of treatment of anemia in low-risk MDS patients and the treatment options in MDS resistant or refractory to ESAs.


Assuntos
Anemia/tratamento farmacológico , Hematínicos/uso terapêutico , Síndromes Mielodisplásicas/sangue , Humanos , Ensaios Clínicos Controlados Aleatórios como Assunto , Fatores de Risco
11.
Med Oncol ; 35(3): 33, 2018 Feb 08.
Artigo em Inglês | MEDLINE | ID: mdl-29417235

RESUMO

Myelodysplastic syndromes (MDS) are heterogeneous clonal disorders ranging from indolent conditions with a near-normal life expectancy to forms approaching acute myeloid leukaemia. Comorbid conditions have rarely been systematically studied among patients with MDS. Older age per se has a negative impact on survival of MDS patients, in particular of those with lower risk. However, age indirectly affects also the survival of higher-risk patients by limiting their eligibility to intensive treatments. In addition, ageing is associated with an increasingly high risk of developing comorbidity, and a high prevalence of comorbid diseases has indeed been reported in MDS patients. The impact of multi-morbidities/comorbidities and polypharmacy in patients with low-risk MDS patients is a poorly explored topic. We focused on medications, multi-morbidities and comorbidities of 155 low-risk MDS patients followed in the haematological outpatients clinics or in medical/oncology wards of our University Hospital. One or more comorbidities were present at diagnosis in 24 younger patients with MDS syndromes (31%), whereas 56 older patients with MDS (75%) presented 1 or more comorbidities (P < 0.001).The most frequent comorbidity was cardiac comorbidity 18% in younger patients and 25% in older patients. With no statistical significance between older and younger patients, congestive heart failure was the most frequent observed disease. Our study has shown a statistical correlation between transfusion dependency and polypathology (P = 0.0014). These data were also confirmed in a subanalysis of the younger group of patients. Our study has shown that comorbidity is very common among patients with MDS, potentially affecting the clinical course and outcome of MDS patients.


Assuntos
Anemia/fisiopatologia , Transfusão de Sangue/estatística & dados numéricos , Comorbidade , Síndromes Mielodisplásicas/epidemiologia , Polimedicação , Índice de Gravidade de Doença , Idoso , Feminino , Seguimentos , Humanos , Itália/epidemiologia , Expectativa de Vida , Masculino , Síndromes Mielodisplásicas/etiologia , Síndromes Mielodisplásicas/terapia , Prevalência , Prognóstico , Medição de Risco
12.
J Thromb Thrombolysis ; 45(1): 106-113, 2018 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-29022213

RESUMO

Arterial and venous complications are major causes of morbidity and mortality in myeloproliferative neoplasms (MPNs). MPNs patients, frequently receive heparin. Heparin-induced thrombocytopenia (HIT) is a rare but potentially life-threatening complication resulting in a severe acquired thrombophilic condition. We carried out a retrospective analysis to evaluate occurrence of new thrombotic events during heparin therapy in essential thrombocythemia (ET) patients. We studied 108 ET patients on heparin for treatment of previous thrombotic events or in thromboprophilaxis. Fifty-eight of them carried JAK 2 V617F mutation while 50 patients were without V617F mutation. Ten patients, among those with JAK 2 V617F mutation after a median of 10 days from heparin treatment presented a platelet drop, new thrombotic events and in 10/10 cases heparin-related antibodies were found. In the other group, two patients (4%) presented a platelet drop, thrombotic manifestations and heparin related antibodies. Our data show that HIT is more frequent, during heparin treatment, in patients with ET carrying V617F mutation, as compared with patients without mutations (P = 0.029). ET with V617F mutation seems to be associated with higher risk of thrombotic complications during heparin treatment. Monitoring platelet counts very closely during the course of heparin is essential especially in ET patients in which platelet drop may be hidden by constitutional thrombocytosis.


Assuntos
Heparina/efeitos adversos , Janus Quinase 2/genética , Mutação de Sentido Incorreto , Trombocitemia Essencial/genética , Trombocitopenia/induzido quimicamente , Trombose/complicações , Adulto , Idoso , Anticorpos/sangue , Feminino , Heparina/imunologia , Humanos , Masculino , Pessoa de Meia-Idade , Contagem de Plaquetas , Prevalência , Estudos Retrospectivos , Trombocitemia Essencial/complicações , Trombocitopenia/classificação , Trombocitopenia/epidemiologia , Trombose/prevenção & controle
13.
Med Oncol ; 35(2): 15, 2017 Dec 29.
Artigo em Inglês | MEDLINE | ID: mdl-29288421

RESUMO

Splenic marginal zone lymphomas (SMZLs) are rare indolent B cell neoplasms that affect the spleen, bone marrow, and blood. Although they have an indolent course in the majority of patients, who have a median survival of 8-10 years, ∼ 30% may experience a worse outcome. The prognostic criteria of progression are lymph node and extra-nodal involvement, high lymphocyte counts, anaemia, and thrombocytopenia. The treatment of SMZLs include a "wait and watch strategy", splenectomy, and alkylating agents ± rituximab. We here describe data relating to 70 patients with intermediate-/high-risk SMZLs, who received rituximab/bendamustine as first-line treatment for a median of 60 days (range 1-75) after diagnosis. Sixty patients (86%) achieved a complete response (CR), and seven (10%) a partial response (PR). Three patients (4.3%) experienced disease progression (PD). The median duration of remission was 18 months. Side effects were generally mild. Our findings suggest that rituximab/bendamustine is a feasible treatment option in patients with intermediate-/high-risk SMZLs.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Linfoma de Zona Marginal Tipo Células B/tratamento farmacológico , Neoplasias Esplênicas/tratamento farmacológico , Idoso , Idoso de 80 Anos ou mais , Cloridrato de Bendamustina/administração & dosagem , Feminino , Seguimentos , Humanos , Linfoma de Zona Marginal Tipo Células B/patologia , Masculino , Prognóstico , Rituximab/administração & dosagem , Neoplasias Esplênicas/patologia
14.
Expert Rev Hematol ; 10(12): 1077-1086, 2017 12.
Artigo em Inglês | MEDLINE | ID: mdl-29069953

RESUMO

INTRODUCTION: Myelodysplastic syndromes (MDS) are a heterogeneous group of hematopoietic disorders characterized by ineffective hematopoiesis and peripheral cytopenia, and their possible transformation into acute myeloid leukemia (AML). They typically affect the elderly but, when making treatment decisions, considering chronological age may be insufficient because it poorly correlates with patient frailty: the challenge is to select the optimal treatment in these patients by balancing efficacy and toxicity. Areas covered: This review discusses the rationale for and methods of personalizing the treatment of elderly MDS patients. Expert commentary: Decisions concerning treatment strategies for elderly MDS patients should be made after assessing their frailty on the basis of a geriatric assessment and an estimate of age-adjusted life expectancy. We suggest that all elderly MDS patients should undergo a timed up and go test (TUGT) as a preliminary means of identifying frail patients, and that all non-frail patients should then undergo a comprehensive geriatric assessment (CGA) in order to distinguish fit and pre-frail patients. Fit patients should receive standard dose treatment; pre-frail patients should receive individualized therapy; and frail patients should receive symptom-related therapy. A repeated CGA may be useful to evaluate the hematological, cognitive and socio-relational effects of MDS treatment.


Assuntos
Síndromes Mielodisplásicas/terapia , Medicina de Precisão , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Tomada de Decisão Clínica , Comorbidade , Gerenciamento Clínico , Avaliação Geriátrica , Transplante de Células-Tronco Hematopoéticas , Humanos , Terapia de Alvo Molecular , Medicina de Precisão/métodos , Medição de Risco , Transplante Homólogo
15.
Ann Hematol ; 96(5): 779-786, 2017 May.
Artigo em Inglês | MEDLINE | ID: mdl-28255868

RESUMO

Anaemia is a complication reported in up to 70% of the multiple myeloma patients (MM), with remarkable clinical, cognitive and socio-relational consequences. Anaemia relates to the course of MM, normalizing in patients during remission and reappearing in relapsing/non-responding patients. In a pilot study with 31 patients with MM and transfusion-dependent anaemia, we evaluated the effects of Binocrit (biosimilar epoetin alfa) on transfusions, haemoglobin levels, mental status (mini-mental state evaluation) and the patients' social-relational functioning and quality of life (QoL). Within a 12-week interval, patients received 40.000 U Binocrit once a week. Binocrit significantly decreased the incidence of transfusion, regardless of the patients' transfusion history, and significantly increased haemoglobin levels (before-and-after-treatment median haemoglobin values = 8.20 vs. 9.40 g/dl, respectively; Wilcoxon Z test, p < .001). A comparatively greater increment in haemoglobin levels among patients who responded to first vs. additional lines of chemotherapy was also observed. Importantly, we additionally found moderate-to-strong positive associations between increments in haemoglobin levels and corresponding increments both in psychological well-being and QoL (FACT-An scores) and the patients' cognitive status (mini-mental state evaluation scores). After statistically controlling for possible concurrent benefits of anti-myeloma therapy, increments in haemoglobin levels clearly predicted both increments in socio-relational FACT-An scores (Spearman's rho = 0.60, p < .001) and in cognitive functioning scores (Spearman's rho = 0.49, p < .006). Binocrit thus appears as an effective, well-tolerated agent for the management of myeloma anaemia, whose documented benefits include amelioration of anaemia, reduction in transfusion, and improvements in the patients' social-relational functioning and cognitive well-being.


Assuntos
Medicamentos Biossimilares/uso terapêutico , Epoetina alfa/uso terapêutico , Hemoglobinas , Mieloma Múltiplo/sangue , Mieloma Múltiplo/terapia , Idoso , Idoso de 80 Anos ou mais , Anemia/sangue , Anemia/etiologia , Anemia/terapia , Transfusão de Sangue , Cognição , Feminino , Humanos , Masculino , Mieloma Múltiplo/complicações , Mieloma Múltiplo/diagnóstico , Projetos Piloto , Qualidade de Vida , Resultado do Tratamento
16.
Ther Adv Med Oncol ; 9(1): 22-32, 2017 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-28203295

RESUMO

BACKGROUND: Many patients with solid tumours or nonmyeloid haematopoietic tumours develop symptomatic anaemia, which has a major impact on quality of life (QoL). The efficacy of erythropoiesis-stimulating agents (ESAs) in improving QoL and reducing blood transfusions has been widely demonstrated. Binocrit® (biosimilar epoetin alfa) is an ESA indicated in the European Union for treating chemotherapy-induced anaemia. The aim of this study was to investigate the effect of Binocrit® on haemoglobin (Hb) levels in anaemic cancer patients in Italian clinical practice. METHODS: The ANEMONE study was a national, longitudinal, retrospective, multicentre observational study. Patients had to be 18 years or older, with a solid tumour or non-Hodgkin's lymphoma, Hodgkin's disease or multiple myeloma, receiving chemotherapy, and treated with Binocrit® to manage chemotherapy-induced anaemia. The primary outcomes were the proportion of patients with a Hb increase ⩾1 g/dl during the first 4 weeks and with a Hb increase ⩾2 g/dl during the first 12 weeks. RESULTS: A total of 245 patients were enrolled and 215 patients were evaluable for statistical analysis. In the first 4 weeks, 49.3% of patients showed an increase in Hb of ⩾1 g/dl: 45.5% in patients with solid tumours and 52.1% in patients with haematological malignancies. In the first 12 weeks, 51.6% of patients showed an increase in Hb of ⩾2 g/dl (48.4% solid tumours, 54.2% haematological diseases). Treatment with Binocrit® was well tolerated. CONCLUSIONS: These results confirm the effectiveness and safety of Binocrit® for chemotherapy-induced anaemia in routine practice in patients with solid tumours, lymphoma and myeloma.

17.
Dig Liver Dis ; 48(11): 1394-1397, 2016 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-27590841

RESUMO

BACKGROUND: Rituximab-containing chemotherapies are offered to elderlies for treatment of non-Hodgkin lymphomas (NHL). From 0.7 to 27% of patients with "resolved" HBV infection develop HBV reactivation and related hepatitis during Rituximab-containing chemotherapies. Currently, several antiviral drugs are available for the prophylaxis of patients at risk for HBV reactivation, which include lamivudine, tenofovir, entecavir, and adefovir. Viral breakthrough may occur during therapy, which is defined as an abrupt increase in serum HBV DNA levels after a period of persistent suppression. Viral breakthrough occurs with non-compliance to therapy and, also, when drug-resistant mutants emerge. The risk might be higher in fragile patients as elderlies. AIMS: Since no study addressed this question, we determined the rate of HBV-RS in patients >65years undergoing Rituximab-containing chemotherapies for NHLs. METHODS: We evaluated 85 newly diagnosed NHL patients with resolved HBV infection, receiving Rituximab-containing chemotherapies. All received lamivudine. HBV DNA was checked at baseline, every 4 weeks, for 1year after completion of Rituximab cointaining regimens. RESULTS: Nine patients (10%) had HBV reactivation and HBV related hepatitis. All received entecavir and recovered without consequences. HBV reactivation was more likely to occur after an average of five R-CHOP cycles or during Fludarabine. CONCLUSIONS: The rate of viral breakthrough (VBK), in our study population, is high considering that the patients were HBV DNA negative at baseline and suggest that Lamivudine prevention may not be sufficient in this population.


Assuntos
Antineoplásicos/uso terapêutico , Hepatite B/epidemiologia , Linfoma não Hodgkin/complicações , Rituximab/uso terapêutico , Ativação Viral , Idoso , Idoso de 80 Anos ou mais , Antivirais/uso terapêutico , DNA Viral/sangue , Feminino , Hepatite B/tratamento farmacológico , Vírus da Hepatite B/isolamento & purificação , Humanos , Itália , Linfoma não Hodgkin/tratamento farmacológico , Masculino , Estudos Retrospectivos
18.
Mediterr J Hematol Infect Dis ; 8(1): e2016030, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27413523

RESUMO

BACKGROUND: Splenic marginal zone lymphoma (SMZL) is a chronic B-cell lymphoproliferative disorder, comprising less than 2% of non-Hodgkin's lymphomas, and affecting mainly middle-aged and elderly patients with a median survival of >10 years. The typical clinical features of SMZL include splenomegaly. Treatment should be patient-tailored and can range from a 'watchful waiting' approach for asymptomatic patients without cytopenias to surgery, localized radiation therapy or immuno/chemotherapies. Recently, the combination of rituximab and Bendamustine (R-Benda) has been defined as highly active in patients with follicular lymphomas, but little is known about the efficacy of R-Benda in SMZL. AIM OF THE STUDY: The purpose of this retrospective study was to report our experience on the efficacy of R-Benda as first line treatment in 23 consecutive elderly SMZL patients. RESULTS: All patients had a complete resolution of splenomegaly along with restoration of their blood counts. Nineteen patients (83%) achieved a complete response (CR) to therapy; three patients (13%) achieved a partial response (PR).Ten patients (43%) obtained molecular remission. Toxicities were mild and mainly haematological and result in dose reductions for fourteen patients. CONCLUSIONS: Our data suggest a high activity and good tolerance of R-Benda, despite dose reduction due to potential toxicity.

19.
Br J Haematol ; 172(6): 902-8, 2016 03.
Artigo em Inglês | MEDLINE | ID: mdl-26728240

RESUMO

Marginal zone lymphoma represents about 10% of all non-Hodgkin lymphomas (NHLs). 33% of patients with acquired angioedema (AAE) due to acquired C1-inhibitor (C1-INH) deficiency (C1-INH-AAE) have or will develop NHLs. C1-INH-AAE is a rare condition. We report the follow-up of 72 C1-INH-AAE patients, followed for a median of 15 years (range 1-24). Median age was 71 (range 64-79) years; median age at onset of angioedema symptoms was 57·5 (range 50-66) years and it was 63 [range 45-80) years at diagnosis]. Twenty patients were diagnosed with low-grade non-follicular B-cell lymphomas (75% were splenic MZL), one with follicular and three with high-grade lymphomas (two diffuse large B-cell lymphomas and one mantle cell lymphoma). Fifteen NHLs were diagnosed at onset of AAE or thereafter (3 months to 7 years), eight had already been diagnosed at onset of angioedema. Two of 24 patients remain on watchful wait. Thirthen of 24 received chemotherapy, two received rituximab. Three underwent splenectomy. All 18 patients receiving therapy for NHL experienced post-treatment reduction in AAE symptoms. Our study suggests that clonal B-cell proliferation is the pathology underlying AAE leading to production of C1-INH-neutralizing autoantibodies and to NHLs. The post-germinal centre origin of NHL suggests that immune stimulation may contribute to lymphomagenesis.


Assuntos
Angioedemas Hereditários/complicações , Linfoma de Zona Marginal Tipo Células B/etiologia , Neoplasias Esplênicas/etiologia , Idoso , Idoso de 80 Anos ou mais , Angioedema/etiologia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Feminino , Seguimentos , Humanos , Linfoma de Zona Marginal Tipo Células B/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Neoplasias Esplênicas/tratamento farmacológico
20.
Clin Chem Lab Med ; 54(9): 1411-26, 2016 Sep 01.
Artigo em Inglês | MEDLINE | ID: mdl-26812791

RESUMO

Myelodisplastic syndromes (MDS) are heterogeneous myeloid disorders characterized by peripheral cytopenias and increased risk of transformation into acute myelogenous leukemia (AML). MDS are generally suspected in the presence of cytopenia on routine analysis and the evaluation of bone marrow cells morphology and cellularity leads to correct diagnosis of MDS. The incidence of MDS is approximately five cases per 100,000 people per year in the general population, but it increases up to 50 cases per 100,000 people per year after 60 years of age. Typically MDS affect the elderly, with a median age at diagnosis of 65-70 years. Here the current therapeutic approaches for MDS are evaluated by searching the PubMed database. Establishing the prognosis in MDS patients is a key element of therapy. In fact an accurate estimate of prognosis drives decisions about the choice and timing of the therapeutic options. Therapy is selected based on prognostic risk assessment, cytogenetic pattern, transfusion needs and biological characteristics of the disease, comorbidities and clinical condition of the patients. In lower-risk patients the goals of therapy are different from those in higher-risk patients. In lower-risk patients, the aim of therapy is to reduce transfusion needs and transformation to higher risk disease or AML, improving the quality of life and survival. In higher-risk patients, the main goal of therapy is to prolong survival and to reduce the risk of AML transformation. Current therapies include growth factor support, lenalidomide, immunomodulatory and hypomethylating agents, intensive chemotherapy, and allogenic stem cell transplantation. The challenge when dealing with MDS patients is to select the optimal treatment by balancing efficacy and toxicity.


Assuntos
Técnicas de Laboratório Clínico , Síndromes Mielodisplásicas/diagnóstico , Síndromes Mielodisplásicas/terapia , Humanos , Síndromes Mielodisplásicas/genética , Medição de Risco
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