Publications by authors named "Rapezzi D"

Article Synopsis
  • The study evaluated the use of frontline TKI therapy in elderly patients (75 years or older) with chronic phase chronic myeloid leukemia (CP-CML) among a large cohort of 332 patients.
  • Results showed that 85.8% of patients received imatinib (IM), while 14.2% were treated with second-generation TKIs (2G-TKI) like dasatinib and nilotinib, with a notable percentage starting on reduced doses.
  • The findings indicated increased usage of IM after generic versions became available in Italy, but significant discontinuation rates due to resistance and toxicities were observed, highlighting the need for personalized treatment assessments and further studies on lower TKI doses.
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BACKGROUND: Whether phlebotomy alone can adequately maintain target hematocrit in patients with low-risk polycythemia vera (PV) remains elusive. METHODS: In a phase 2 open-label randomized trial, we compared ropeginterferon alfa-2b (ropeg; 100 μg every 2 weeks) with phlebotomy only regarding maintenance of a median hematocrit level (≤45%) over 12 months in the absence of progressive disease (primary end point). In follow-up, crossover to the alternative treatment group was allowed if the primary end point was not met.

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Data on the efficacy of high-dose chemotherapy and autologous stem cell transplantation (ASCT) for classical Hodgkin lymphoma (cHL) patients who failed a PET-driven first-line therapy are limited.We retrospectively evaluated 220 adult cHL patients who underwent ASCT from 2009 to 2021 at 11 centers in Italy. Overall, 49.

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In patients with low-risk polycythemia vera, exposure to low-dose Ropeginterferon alfa-2b (Ropeg) 100 µg every 2 weeks for 2 years was more effective than the standard treatment of therapeutic phlebotomy in maintaining target hematocrit (HCT) (< 45%) with a reduction in the need for phlebotomy without disease progression. In the present paper, we analyzed drug survival, defined as a surrogate measure of the efficacy, safety, adherence, and tolerability of Ropeg in patients followed up to 5 years. During the first 2 years, Ropeg and phlebotomy-only (Phl-O) were discontinued in 33% and 70% of patients, respectively, for lack of response (12 in the Ropeg arm vs.

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Polycythemia vera (PV) is a myeloproliferative neoplasm characterized by aberrant myeloid lineage hematopoiesis with excessive red blood cell and pro-inflammatory cytokine production. Patients with PV present with a range of thrombotic and hemorrhagic symptoms that affect quality of life and reduce overall survival expectancy. Thrombotic events, transformation into acute myeloid leukemia, and myelofibrosis are largely responsible for the observed mortality.

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Article Synopsis
  • - In advanced systemic mastocytosis (AdvSM), cytoreductive treatment is becoming more diverse, particularly with new medications like KIT tyrosine kinase inhibitors.
  • - Avapritinib, a second-generation drug specifically targeting the D816V mutation, has gained approval from US FDA and EMA for treating adults with AdvSM based on successful clinical trial results.
  • - An Italian compassionate use program showed that avapritinib is effective for patients who have undergone multiple treatments, providing significant responses and potentially helping as a temporary treatment before an allogeneic transplant, though careful monitoring for side effects is necessary.
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Background: Imatinib, dasatinib, and nilotinib are tyrosine kinase inhibitors (TKIs) approved in Italy for frontline treatment of chronic-phase chronic myeloid leukemia (CP-CML). The choice of TKI is based on a combined evaluation of the patient's and the disease characteristics. The aim of this study was to analyze the use of frontline TKI therapy in an unselected cohort of Italian patients with CP-CML to correlate the choice with the patient's features.

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We describe a case of concomitant erythropoietin allergy and resistance with a possible IgE and IgG-mediated immune response, in which the local allergic cutaneous symptoms preceded the antibody-mediated anemia.

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Article Synopsis
  • Understanding antibody responses to SARS-CoV-2 in hematologic malignancy (HM) patients post-infection is vital for tailoring vaccination strategies for this vulnerable group.
  • A study of 189 HM patients showed an 85.7% overall seroconversion rate, with lower rates in patients with lymphoid malignancies or those undergoing chemotherapy.
  • Therapy-naive patients ("watch and wait") were more likely to seroconvert and had higher anti-S IgG levels, while factors like male gender and severe COVID-19 also correlated with improved neutralizing activity.
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Atypical Chronic Myeloid Leukemia, BCR-ABL1 negative (aCML) is a rare hematological entity, included in the group of myelodysplastic (MDS)/myeloproliferative (MPN) overlap syndromes. It is characterized by an aggressive course, a high rate of acute myeloid leukemia (AML) transformation, and a dismal outcome. The clinical presentation includes splenomegaly and leukocytosis with neutrophilia and left-shifted granulocytosis accompanied by granulocytic dysplasia and sometimes multilineage dysplasia.

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Limited information is available on the impact of the COVID-19 pandemic on the management of chronic myeloid leukaemia (CML). The Campus CML network collected retrospective information on 8 665 CML patients followed at 46 centres throughout Italy during the pandemic between February 2020 and January 2021. Within this cohort, we recorded 217 SARS-CoV-2-positive patients (2·5%).

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Article Synopsis
  • Jak inhibitors are strong anti-inflammatory medications that may help reduce the severe inflammatory response in COVID-19 patients with pneumonia.
  • A study involving 218 hospitalized patients showed that 66.5% experienced improvement after treatment with ruxolitinib, particularly within the first week.
  • By the end of the observation, 87.2% of patients survived, with some experiencing significant improvements in breathing and inflammatory markers, and minimal serious side effects were reported.
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Abnormal NIPT results, contrasting with normal fetus development, could disclose maternal malignancy, and this possibility should always be explained during pretest counseling. In this case, a complete diagnostic assessment is recommended and should be managed by a multidisciplinary team to define the best timing for diagnostic procedures, delivery, and treatment.

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Background: There is no evidence that phlebotomy alone is sufficient to steadily maintain haematocrit on target level in low-risk patients with polycythaemia vera. This study aimed to compare the efficacy and safety of ropeginterferon alfa-2b on top of the standard phlebotomy regimen with phlebotomy alone.

Methods: In 2017, we launched the Low-PV study, a multicentre, open-label, two-arm, parallel-group, investigator-initiated, phase 2 randomised trial with a group-sequential adaptive design.

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  • Immunoglobulin M multiple myeloma and Waldenström macroglobulinemia are distinct but overlapping blood disorders characterized by the presence of immunoglobulin M monoclonal gammopathy.
  • Two cases highlight the importance of accurate diagnosis despite similar bone marrow presentation; one patient had multiple myeloma with different treatments compared to the other's diagnosis of Waldenström macroglobulinemia.
  • Differentiating between these conditions is crucial for appropriate treatment, emphasizing the need for thorough diagnostic evaluations, including cytogenetics and mutation testing.
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Several papers authored by international experts have proposed recommendations on the management of BCR-ABL1+ chronic myeloid leukemia (CML). Following these recommendations, survival of CML patients has become very close to normal. The next, ambitious, step is to bring as many patients as possible into a condition of treatment-free remission (TFR).

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Patients with Philadelphia-negative myeloproliferative neoplasm (MPN) are prone to the development of second cancers, but the factors associated with these events have been poorly explored. In an international nested case-control study, we recruited 647 patients with carcinoma, nonmelanoma skin cancer, hematological second cancer, and melanoma diagnosed concurrently or after MPN diagnosis. Up to 3 control patients without a history of cancer and matched with each case for center, sex, age at MPN diagnosis, date of diagnosis, and MPN disease duration were included (n = 1234).

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One out of ten patients with Philadelphia-negative myeloproliferative neoplasms (MPN) develop a second cancer (SC): in such patients we aimed at assessing the survival impact of SC itself and of MPN-specific therapies. Data were therefore extracted from an international nested case-control study, recruiting 798 patients with SC diagnosed concurrently or after the MPN. Overall, 2995 person-years (PYs) were accumulated and mortality rate (MR) since SC diagnosis was 5.

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Article Synopsis
  • A study involving 293 Italian patients with chronic myeloid leukemia (CML) showed that discontinuing tyrosine kinase inhibitors (TKI) after reaching a deep molecular response is generally safe, with an overall estimated treatment-free remission (TFR) rate of 62% after a median follow-up of 34 months.
  • The majority of patients were using imatinib (72%), with fewer on second-generation TKIs (28%); those on second-generation TKIs had shorter treatment durations compared to imatinib.
  • Most patients (88%) stopped treatment based on clinical practice, primarily due to shared decisions or toxicity, with no relapses reported during the study period.
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We analyzed 597 patients with myeloproliferative neoplasms (MPN) who presented transient ischemic attacks (TIA, n = 270) or ischemic stroke (IS, n = 327). Treatment included aspirin, oral anticoagulants, and cytoreductive drugs. The composite incidence of recurrent TIA and IS, acute myocardial infarction (AMI), and cardiovascular (CV) death was 4.

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Purpose To investigate the progression-free survival (PFS) of patients with advanced Hodgkin lymphoma (HL) after a risk-adapted treatment strategy that was based on a positive positron emission tomography scan performed after two doxorubicin, vinblastine, vincristine, and dacarbazine (ABVD) cycles (PET2). Patients and Methods Patients with advanced-stage (IIB to IVB) HL were consecutively enrolled. After two ABVD cycles, PET2 was performed and centrally reviewed according to the Deauville five-point scale.

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Purpose: The main objective of this study is to gain a deeper understanding of how patients suffering from chronic myeloid leukemia (CML) cope with their illness. The study aims to reconstruct the subjective meaning-making process related to CML in order to gain insights into the impact the disease has on patients' emotions and everyday lives, as well as to explore the psychological impact of their being presented with the chance to suspend their therapy and recover from the disease.

Methods: Data were gathered from a qualitative study conducted in Italy on 158 Italian CML patients.

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We retrospectively studied 181 patients with polycythaemia vera (n=67), essential thrombocythaemia (n=67) or primary myelofibrosis (n=47), who presented a first episode of splanchnic vein thrombosis (SVT). Budd-Chiari syndrome (BCS) and portal vein thrombosis were diagnosed in 31 (17.1%) and 109 (60.

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