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Managing Relevant Clinical Conditions of Hemophilia A/B Patients

Hematol. Rep. 2023, 15(2), 384-397; https://doi.org/10.3390/hematolrep15020039

The Medical Directors of nine Italian Hemophilia Centers reviewed and discussed the key issues concerning the replacement therapy of hemophilia patients during a one-day consensus conference held in Rome one year ago. Particular attention was paid to the replacement therapy needed for surgery using continuous infusion (CI) versus bolus injection (BI) of standard and extended half-life Factor VIII (FVIII) concentrates in severe hemophilia A patients. Among the side effects, the risk of development of neutralizing antibodies (inhibitors) and thromboembolic complications was addressed. The specific needs of mild hemophilia A patients were described, as well as the usage of bypassing agents to treat patients with high-responding inhibitors. Young hemophilia A patients may take significant advantages from primary prophylaxis three times or twice weekly, even with standard half-life (SHL) rFVIII concentrates. Patients affected by severe hemophilia B probably have a less severe clinical phenotype than severe hemophilia A patients, and in about 30% of cases may undergo weekly prophylaxis with an rFIX SHL concentrate. The prevalence of missense mutations in 55% of severe hemophilia B patients allows the synthesis of a partially changed FIX molecule that can play some hemostatic role at the level of endothelial cells or the subendothelial matrix. The flow back of infused rFIX from the extravascular to the plasma compartment allows a very long half-life of about 30 h in some hemophilia B patients. Once weekly, prophylaxis can assure a superior quality of life in a large severe or moderate hemophilia B population. According to the Italian registry of surgery, hemophilia B patients undergo joint replacement by arthroplasty less frequently than hemophilia A patients. Finally, the relationships between FVIII/IX genotypes and the pharmacokinetics of clotting factor concentrates have been investigated.
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Extracellular Vesicles and Their Role in Patients with Multiple Myeloma

Hematol. Rep. 2021, 13(s2), 1-7; https://doi.org/10.3390/hematolrep13120001

B cell lymphoproliferative diseases, including Multiple Myeloma [...]

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1st SOHO Clinical and Biological School of Acute Leukemia

Hematol. Rep. 2021, 13(s1), 1-13; https://doi.org/10.3390/hematolrep13110001

Genomic and functional alterations of enzymatic activity drive cancer metabolic reprogramming along with mutations of tumor suppressors and oncogenes [...]
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1st SOHO Italy, Clinical and Biological School of Multiple Myeloma

Hematol. Rep. 2020, 12(s2), 1-24; https://doi.org/10.3390/hematolrep12120001

The broad use of immunomodulatory drugs (IMiDs) and the breakthrough of novel immunotherapies in MM [...]
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Abstract Book of the SOHO 3rd Italian Conference | 27–30 September 2021

Hematol. Rep. 2021, 13(3), 9389; https://doi.org/10.4081/hr.2021.9389

Bruton’s tyrosine kinase (BTK) inhibition is an emerging standard of care for Waldenström macroglobulinemia [...]
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How I Manage Systemic Light Chain Amyloidosis

Hematol. Rep. 2021, 13(3), 9383; https://doi.org/10.4081/hr.2021.9383

Light chain amyloidosis (AL) is a protein deposition disorder with a heterogenous pattern of organ involvement and dysfunction that varies by affected patient. Often diagnosed late after the onset of symptoms, appropriate and correct diagnosis, and prompt initial management, typically with anti-plasma cell therapy targeting the underlying cell producing the aberrant light chain protein, can lead to significant improvement in patient symptoms, organ function and lifespan. With recent publication of phase III studies focused on AL, and a changing regulatory landscape providing greater availability of novel therapies, the management of AL is in some ways more complex with greater options to consider. In this clinical review, a discussion of the diagnosis, staging, and goals of therapy transitions to a focus on contemporary management questions to outline our clinical approach to multi-disciplinary management and long term follow up for patients with suspected or confirmed AL.
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Advancing Leukemia Diagnostics: Role of Next Generation Sequencing (NGS) in Acute Myeloid Leukemia

Hematol. Rep. 2020, 12(s1), 8957; https://doi.org/10.4081/hr.2020.8957

AML diagnostics, initially based solely on morphological evaluation, now relies on multiple disciplines to reach its full potential. Only by integrating the results of cytomorphology, cytochemistry, immunophenotyping, cytogenetics and molecular genetics it is possible to fulfil WHO classification and ELN prognostication systems. Especially molecular genetics has gained a lot of interest over the last decade, mainly through the introduction of next generation sequencing (NGS). NGS application ranges from the investigation of single genes and panels to even whole exomes, transcriptomes and genomes. In routine AML diagnostics panels are the preferred NGS methodology. Here, we will review the power and limitations of NGS in the context of diagnosis, prognosis and precision medicine. Due to high dimensionality, NGS data interpretation is challenging but it also offers a unique investigatory chance and the opportunity to apply data mining techniques such as artificial intelligence. We will also reflect on how the incorporation of the improved knowledge base into routine diagnostics can pave the way for better treatment and more cure in AML.
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Treatment-Free Remission in Chronic Myeloid Leukemia: Lights and Shadows

Hematol. Rep. 2020, 12(s1), 8950; https://doi.org/10.4081/hr.2020.8950

In addition to the best possible overall survival, discontinuation of the tyrosine kinase-inhibitor (TKI) treatment [treatment free remission (TFR)] without observing a recurrence of the disease has become a standard part of chronic myeloid leukemia (CML) care. Worldwide, more than 2000 patients with CML have attempted TFR, and very rare instances of disease transformation have been reported. Several studies in the last decade have demonstrated the feasibility and safety of TKI discontinuation in selected patients with CML who achieve deep and sustained molecular response with TKI. This has moved prime-time into clinical practice although open questions remain in terms of understanding the disease biology that leads to successful TKI cessation in some patients while not in others. Despite the remaining questions regarding which factors may be considered predictive for TFR, treatment interruption is a safe option provided that adequate molecular monitoring is available, with prompt re-initiation of TKIs as soon as major molecular response has been lost. Data from ongoing trials should help refine decisions as to which patients are the best candidates to attempt TKI discontinuation, frequency of a safe monitoring, optimal strategies to sustain ongoing TFR and increase the number of patients who can access to discontinuation programs.
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How We Manage Smoldering Multiple Myeloma

Hematol. Rep. 2020, 12(s1), 8951; https://doi.org/10.4081/hr.2020.8951

Smoldering myeloma (SMM) is an asymptomatic stage characterized by bone marrow plasma cells infiltration between 10–60% in absence of myeloma-defining events and organ damage. Until the revision of criteria of MM to require treatment, two main prognostic models, not overlapping each other, were proposed and used differently in Europe and in US. Novel manageable drugs, like lenalidomide and monoclonal antibodies, with high efficacy and limited toxicity, improvement in imaging and prognostication, challenge physicians to offer early treatment to high-risk SMM. Taking advantage from the debates offered by SOHO Italy, in this review we will update the evidence and consequent clinical practices in US and Europe to offer readers a uniform view of clinical approach at diagnosis, follow-up and supportive care in the SMM setting.
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How I Treat Relapsed and/or Refractory Multiple Myeloma

Hematol. Rep. 2020, 12(s1), 8955; https://doi.org/10.4081/hr.2020.8955

The expanding therapeutic landscape of relapsed and/or refractory multiple myeloma (RRMM) has contributed to significant improvements in patient outcomes. These have included combinations of proteasome inhibitors (PIs), immunomodulatory drugs (IMiDs), monoclonal antibodies (mAbs), histone deacetylase inhibitors, and/or alkylating agents. More recently, the approval of the first-in-class nuclear export inhibitor selinexor and the first-in-class B-cell maturation antigen (BCMA) antibody-drug conjugate (ADC) belantamab mafodotin has helped address the current unmet need in patients refractory to PI, IMiD, and anti-CD38 mAb directed therapy, otherwise known as triple class refractory myeloma. With the growing number of treatment options in the RRMM therapeutic landscape, the choice and sequencing of drugs and combinations has become increasingly complex. In this review we discuss our approach and considerations in the treatment of both early and late RRRM based on best available data and our clinical experience.
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How I Manage Frontline Transplant-Eligible Multiple Myeloma in Italy

Hematol. Rep. 2020, 12(s1), 8954; https://doi.org/10.4081/hr.2020.8954

The treatment of transplant-eligible multiple myeloma patients in Italy consists in an induction phase based on bortezomib plus thalidomide plus dexamethasone (VTd), followed by a single or tandem autologous stem cell transplantation (ASCT), followed by lenalidomide maintenance. This approach offers an overall response rate of 93% and a CR rate of 58% with acceptable toxicity. Lenalidomide maintenance adds a significant increase in disease control, with a progression free survival after ASCT of 53 months, and an overall survival of 86 months. Second primary malignancies represent the most concerning toxicity of lenalidomide maintenance with a 6.9% incidence. However, the benefit in terms of increased myeloma control largely outweigh this complication. The incorporation of daratumumab in this treatment schema will further improve these clinical results.
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How I Manage Frontline Transplant-Ineligible Multiple Myeloma

Hematol. Rep. 2020, 12(s1), 8956; https://doi.org/10.4081/hr.2020.8956

The Multiple Myeloma (MM) is a plasma cells hematological malignancy with a median age of 69 years at diagnosis. The autologous stem cell transplantation is the standard of care for this disease but less than half of newly diagnosed patients are assessed for this treatment due to comorbidities or complications of disease. The management of transplant ineligible MM patients is based on the balance safety and efficacy of the new available regimen and a careful assessment of the frailty status is mandatory to define the goals. In this review we discuss of the clinical dilemmas in the management and define how to manage them based on the evidence from clinical trials and “real life” experience.
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Abstract Book—XLIV Congresso Nazionale AIEOP | 13–15 Ottobre 2019, Catania (Italy)

Hematol. Rep. 2019, 11(s1), 8337; https://doi.org/10.4081/hr.2019.8337

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XLIII Congresso Nazionale AIEOP|Bologna, 27–29 Maggio 2018

Hematol. Rep. 2018, 10(s1), 7767; https://doi.org/10.4081/hr.2018.7767

INTRODUZIONE: Gli attuali protocolli chemioterapici e il miglioramento delle tecniche chirurgiche e radioterapiche hanno aumentato le aspettative di vita delle bambine affette da patologie neoplastiche [...]
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XLII Congresso Nazionale AIEOP | San Giovanni Rotondo, 21–23 maggio 2017—Abstract Book

Hematol. Rep. 2017, 9(s1), 7231; https://doi.org/10.4081/hr.2017.7231

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XLI Congresso Nazionale AIEOP | Verona, 22–24 Maggio 2016 - Abstract Book

Hematol. Rep. 2016, 8(s1), 6611; https://doi.org/10.4081/hr.2016.6611

l MMF è un immunosoppressore che riduce la pro-liferazione dei linfociti T/B e che si è dimostrato utile in alcuni pazienti con PTI e con SE secondaria ad ALPS [...]
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7th International Symposium on Clinical Applications of Free Light Chain and Heavy/Light Chain Analysis

Hematol. Rep. 2015, 7(1s), 5946; https://doi.org/10.4081/hr.2015.5946

Background. Quantification of monoclonal IgM (M-IgM) using electrophoretic techniques can be challenging [...]
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10th International Hereditary Hemorrhagic Telangiectasia Scientific Conference, 12-15 June 2013 - Cork, Ireland

Hematol. Rep. 2013, 5(s1), s1; https://doi.org/10.4081/hr.2013.s1

We are arguably entering a new era in scientific research. The availability of novel research tools, such as the human genome sequence and inexpensive genome sequencing have the potential not only to provide new insights into the fundamental biology of many conditions, but, literally, to redefine health and disease [...]
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10th International Winter Meeting on Coagulation, Basic, Laboratory and Clinical Aspects of Venous and Arterial Thromboembolic Diseases, Bormio, Italy, 10-16 April 2011

Hematol. Rep. 2011, 3(s1), s1; https://doi.org/10.4081/hr.2011.s1

Activation of platelets leads to an increase in the concentration of cytosolic calcium (Ca++) which is a major component of the signaling mechanism regulating platelet function [...]
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9th International Hereditary Hemorrhagic Telangiectasia Scientific Conference, 20–24 May 2011 Kemer, Antalya—Turkey

Hematol. Rep. 2011, 3(s2), s2; https://doi.org/10.4081/hr.2011.s2

HHT shows considerable clinical variability in severity of phenotypes even within families harboring the same pathogenic mutation, suggestive of environmental and/or genetic factors that modify the penetrance and expressivity of the clinical manifestatio [...]
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6th International Symposium on Clinical Applications of Serum Free Light Chain Analysis (plus Hevylite)

Hematol. Rep. 2010, 2(s2), 2062; https://doi.org/10.4081/2062

The 6th International Symposium on Clinical Applications of Serum Free Light Chain Analysis (plus Hevylite) was held in Bath, UK on 23rd and 24th September 2010. This Symposium included measurements in monoclonal gammopathies, myeloma kidney and inflammatory diseases; data from the Multi-centre, Phase III, Randomised control trial on extended haemodialysis with the Gambro protein-leaking HCO 1100 dialyser; and presentations on Hevylite (intact immunoglobulin kappa/lambda ratios) in a variety of clinical situations.
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XXXVI Italian Congress of AIEOP

Hematol. Rep. 2010, 2(s1), 1912; https://doi.org/10.4081/1912

Le Anemie neonatali sono caratterizzate dalla riduzione dell’emoglobina (Hb) o dell’ematocrito (Ht) al di sotto dei valori normali per l’età (<2 DS); la definizione dello stato anemico, anche in questa particolare epoca della vita, necessita di una corretta interpretazione dei dati forniti dall’esame emocromocitometrico, in rapporto ai valori propri dell’età (Tabelle 1–2). [...]
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