Publications by authors named "Novella Pugliese"

Daratumumab combined with bortezomib, thalidomide, and dexamethasone (Dara-VTD) is a highly effective induction therapy for newly diagnosed multiple myeloma (NDMM) patients eligible for autologous stem cell transplantation (ASCT). However, its impact on stem cell mobilization requires a critical evaluation. This study examines the effects of Dara-VTD on stem cell mobilization and collection outcomes.

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Therapeutic phlebotomy remains a key intervention in the management of erythrocytosis and iron overload disorders, particularly polycythemia vera (PV) and hereditary hemochromatosis. Despite its historical origins as an ancient practice, venesection continues to be recommended in international guidelines for the reduction of hematocrit and iron burden, thereby mitigating thrombotic and organ-related complications. However, the evolving landscape of targeted pharmacologic therapies is reshaping the therapeutic paradigm.

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Background: The response to ruxolitinib after 6 months (RR6) model allows early identification of ruxolitinib-treated myelofibrosis (MF) patients with poorer overall survival (OS); however, it is less applicable to lower-risk patients.

Methods: To further explore this, the authors performed a subanalysis of the "RUX-MF" study (NCT06516406) with an aim to validate the RR6 and to develop a score specific for intermediate-1 DIPSS/MYSEC-PM risk patients.

Results: Among the 776 evaluable patients, 34.

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Polycythemia vera (PV) is a chronic myeloproliferative neoplasm with a substantial risk of thromboembolic events (TEs), which contribute to morbidity and mortality. Traditional thrombotic risk stratification primarily considers age and thrombosis history, yet these parameters alone do not capture the complexity of thrombotic risk. Growing evidence highlights the role of additional factors influencing the risk of TEs, underscoring the need for a more comprehensive approach to patient stratification.

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Introduction: Excisional biopsy (EB) is the gold standard for large B-cell lymphomas (LBCLs) diagnosis. Based on recent advances in interventional radiology enabling accurate sampling with core needle biopsy (CNB), we evaluated efficacy and safety of CNB under imaging guidance for diagnosing LBCLs.

Methods: At the Hematology and Pathology Units of the Federico II University Medical School of Naples (Italy), we retrospectively collected patients with lymphadenopathies suspected of lymphomas (during 2009-2022) of which the ultrasonography (US)-guided CNB lymph node samples were available.

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Background/objectives: Lymph node (LN) evaluation is critical in diagnosing, staging, and managing various diseases, particularly lymphoma and metastatic cancer. Although conventional ultrasound (US) is widely used for this purpose, its limitations in reliably differentiating between benign and malignant LNs persist. Ultrasound elastography (US-E), which evaluates tissue stiffness, has emerged as a promising adjunct to improve diagnostic accuracy.

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Introduction: In patients with myelofibrosis (MF), overall survival (OS) after ruxolitinib discontinuation is poor, with leukemic transformation, clonal evolution and thrombocytopenia as the main factors worsening prognosis.

Patients And Methods: To assess the impact of disease phenotype on outcome after ruxolitinib discontinuation in chronic phase patients, we performed a sub-analysis of the "RUX-MF" study (NCT06516406), which now includes 1055 MF patients who received ruxolitinib in a real-life context.

Results: After a median follow-up of 3.

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Splenomegaly is a quite common clinical feature of Philadelphia (Ph) negative chronic myeloproliferative neoplasms (MPNs) and its presence may, in some cases, drives treatment decision. Most importantly, palpable splenomegaly is a minor criterion for both pre-fibrotic/early primary myelofibrosis and primary myelofibrosis (PMF) diagnosis, even if clinical assessment by physical examination is poorly reliable and accurate. On the other hand, despite the International Working Group-Myeloproliferative Neoplasms Research and Treatment and European LeukemiaNet guidelines defined spleen response criteria by palpation, they also recognized the highly subjective nature of spleen size assessment by physical examination, and recommended objective confirmation of volume reduction via computed tomography or magnetic resonance imaging (MRI).

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Calreticulin (CALR) mutations are detected in around 20% of patients with primary and post-essential thrombocythemia myelofibrosis (MF). Regardless of driver mutations, patients with splenomegaly and symptoms are generally treated with JAK2-inhibitors, most commonly ruxolitinib. Recently, new therapies specifically targeting the CALR mutant clone have entered clinical investigation.

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Article Synopsis
  • Ruxolitinib (RUX) is a treatment for myelofibrosis, mainly studied in high-risk patients, but is often given to intermediate-1 patients with limited data on its effects.
  • In a study of 1,055 myelofibrosis patients, over half were classified as intermediate-1 risk, with notable symptoms and some having high-molecular-risk mutations.
  • The study found that after 6 months of RUX treatment, a significant proportion of patients experienced improvements in spleen size and symptoms, with certain factors like the absence of high-molecular-risk mutations being linked to better treatment responses.
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  • Anemia is common in patients with myelofibrosis (MF) and is worsened by the treatment drug ruxolitinib (RUX), with new cases of blast phase (BP) emerging in anemic patients not previously on this treatment.
  • A study of 886 MF patients treated with RUX found a BP incidence rate of 3.74 per 100 patient-years, with higher rates in patients who had varying levels of anemia; the most severe cases were in those dependent on transfusions.
  • The findings suggest that both pre-existing and treatment-induced anemia significantly increase the risk of BP development, indicating a need for better anemia treatments alongside MF therapies.
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  • A significant number of myelofibrosis patients stop using ruxolitinib within the first 5 years due to treatment failure, highlighting the need for early identification of those at risk.* -
  • A study analyzed data from 889 patients and found that factors like low platelet count, low hemoglobin, and certain disease types increase the likelihood of stopping ruxolitinib treatment early.* -
  • A new prognostic model called STR-PM was developed to categorize patients into low, intermediate, and high-risk groups for early treatment failure, suggesting that those in higher risk categories might benefit from alternative treatments.*
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Cyclic neutropenia is a rare hematological condition characterized by periodic fluctuations in neutrophil counts, with a 21-day periodicity. Clinical presentation varies from mild to severe forms of the disease, with the onset of recurrent fever, painful oral ulcers, recurrent bacterial infections, peritonitis, and septic shock. The availability of granulocyte colony-stimulating factor (G-CSF) has revolutionized the management and natural history of this disease, regulating the proliferation, differentiation, and maturation of the progenitor cells, and reducing the duration of neutropenia.

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Article Synopsis
  • * Factors linked to achieving a complete response included the absence of splenomegaly, experiencing pruritus, and having a higher hydroxyurea dosage; however, higher doses also led to more side effects.
  • * Despite many patients having a partial or no response, a significant portion continued on hydroxyurea rather than switching to ruxolitinib, with symptoms like splenomegaly motivating the switch; the study suggests improvements in hydroxy
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The reliability and safety of front-line ultrasonography guided core needle biopsy (UG-CNB) performed with specific uniform approach have never been evaluated in a large series of patients with lymphadenopathies suspected of lymphoma. The aim of this study was to assess the overall accuracy of UG-CNB in the lymph node histological diagnosis, using a standard reference based on pathologist consensus, molecular biology, and/or surgery. We retrospectively checked the findings concerning the application of lymph node UG-CNB from four Italian clinical units that routinely utilized 16-gauge diameter modified Menghini needle under power-Doppler ultrasonographic guidance.

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The basilic/brachial (BBV), internal jugular (IJV), and subclavian veins (SCV) are commonly used as central venous catheter (CVC) sites. A BBV approach [peripherally inserted central catheter (PICC)] is increasingly used for short- to intermediate-term CVCs for acute leukemias undergoing cytotoxic intensive regimens. In this retrospective study, the catheterization of the BBV, IJV, and SCV in patients with previously untreated acute leukemia was assessed.

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Article Synopsis
  • Patients with cytopenic myelofibrosis (MF) face more challenges in treatment options and prognosis compared to those with a myeloproliferative phenotype, with common definitions for cytopenia including low leukocyte, hemoglobin, or platelet counts.
  • In a study of 886 patients treated with ruxolitinib, 45.9% had cytopenic MF and factors like high molecular risk mutations and high prognostic scores were linked to this phenotype.
  • Patients with cytopenia received lower doses of ruxolitinib and had poorer response rates for symptoms and spleen size, along with a higher likelihood of treatment discontinuation over five years compared to those
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Introduction: Occult hepatitis B infection (OBI) is a condition where replication-competent hepatitis B virus-DNA (HBV-DNA) is present in the liver, with or without HBV-DNA in the blood [<200 international units (IU)/ml or absent] in HB surface antigen (HBsAg)-negative/HB core antibody (HBcAb)-positive individuals. In patients with advanced stage diffuse large B-cell lymphoma (DLBCL) undergoing 6 cycles of R-CHOP-21+2 additional R, OBI reactivation is a frequent and severe complication. There is no consensus among recent guidelines on whether a pre-emptive approach or primary antiviral prophylaxis is the best solution in this setting of patients.

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  • Erythropoiesis-stimulating agents (ESA), particularly biosimilars (B-ESA), show promise in treating anemia in myelofibrosis (MF), although their effectiveness is not fully established.
  • A study of 96 MF patients indicated an 80% anemia response rate with B-ESA, where important factors for response included being transfusion-independent and having low ferritin levels at the start of treatment.
  • The treatment was generally well-tolerated, with only 2.5% discontinuing due to toxicity, and those who responded to B-ESA had better survival rates compared to non-responders, suggesting the need for further research on its clinical benefits.
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Systemic mastocytosis (SM) is a rare disease with a range of clinical presentations, and the vast majority of patients have a KIT D816V mutation that results in a gain of function. The multikinase/KIT inhibitor midostaurin inhibits the D816V mutant and has a well-established role in treating advanced SM. Even if considered the standard of therapy, some open questions remain on optimizing midostaurin management in daily practice.

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  • Polycythemia vera (PV) is a disorder that leads to an overproduction of red blood cells and has diverse treatment approaches, including the use of ruxolitinib as a secondary option for patients not responding to hydroxyurea.
  • A survey conducted with 60 hematologists and transfusion specialists in Italy aimed to understand their treatment practices for PV patients, focusing on different patient conditions and therapy options.
  • The findings revealed significant variability in how PV is managed by clinicians, indicating a need for further research to develop clearer guidelines for treating this condition.
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We evaluated the impact of liposomal doxorubicin (NPLD) supercharge-containing therapy on interim fluorodeoxyglucose positron emission tomography (interim-FDG-PET) responses in high-risk diffuse large B-cell lymphoma (DLBCL) or classical Hodgkin lymphoma (c-HL). In this phase II study (2016-2021), 81 adult patients with advanced-stage DLBCL (n = 53) and c-HL (n = 28) received front-line treatment with R-COMP-dose-intensified (DI) and MBVD-DI. R-COMP-DI consisted of 70 mg/m of NPLD plus standard rituximab, cyclophosphamide, vincristine and prednisone for three cycles (followed by three cycles with NPLD de-escalated at 50 mg/m ); MBVD-DI consisted of 35 mg/m of NPLD plus standard bleomycin, vinblastine and dacarbazine for two cycles (followed by four cycles with NPLD de-escalated at 25 mg/m ).

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Contrast-enhanced ultrasonography (CEUS) use for detecting lymphoma in the spleen was questioned because of the risk of its inadequate diagnostic accuracy. The aim of the present study was to validate CEUS exam for the identification of spleen involvement by lymphoma in patients at risk. A total of 260 nodules from the spleens of 77 patients with lymph node biopsy-proven non-Hodgkin lymphoma (NHL; n = 44) or Hodgkin lymphoma (HL; n = 33) at staging (n = 56) or follow-up (n = 21) were collected in a hematology Italian center and retrospectively analyzed.

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