Deferasirox in the management of iron‐overload in patients with myelofibrosis: a multicentre study from the Rete Ematologica Lombarda (<scp>IRON</scp>‐M study)

Elli, Elena Maria; Iurlo, Alessandra; Caramella, Marianna; Malato, Simona; Casartelli, Elena; Maffioli, Margherita; Gardellini, Angelo; Carraro, Maria Cristina; D’Adda, Mariella; Polverelli, Nicola; Rossi, Marianna; Orofino, Nicola; Carrer, Andrea; Gambacorti‐Passerini, Carlo; Antolini, Laura; Passamonti, Francesco

doi:10.1111/bjh.15964

Cited by 11 publications

(16 citation statements)

References 15 publications

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“…The results show that in both studies, patients who obtained an ICR experienced a progressive reduction in ferritin levels at 6, 12, and 18 months, compared to non-responders (68). As regards the identification of factors predictive of an ICR to deferasirox, in the study by Elli et al (68), patients who obtained efficacious ICR presented statistically lower ferritin levels at baseline than nonresponders. Similarly, the median number of prior transfusions and the duration of the history of transfusion dependence were statistically lower/shorter amongst responders.…”

Section: Impact Of Deferasirox Treatment In Reducing Ferritin and Inducing Erythroid Response In Transfusion-dependent Myelofibrosismentioning

confidence: 86%

“…Therefore, by reducing the IOL, treatment with deferasirox seems to provide a significant advantage in terms of the survival in patients with MF. The rate of leukemia evolution or progression disease seems to be lower in ICR group, with a better 2-year leukemia free survival (LFS, p=0.039) (68), but this result needs to be confirmed in a larger and prospective cohort.…”

Section: Impact Of Deferasirox Treatment In Reducing Ferritin and Inducing Erythroid Response In Transfusion-dependent Myelofibrosismentioning

confidence: 89%

“…These data highlight that an earlier access to iron chelation therapy constitutes an advantage in terms of efficacious iron chelation. On the other hand, other than the statistically significant relationship between the achievement of ICR and the obtainment of hematological improvement, it was not possible to identify factors predictive of hematological response (68).…”

Section: Impact Of Deferasirox Treatment In Reducing Ferritin and Inducing Erythroid Response In Transfusion-dependent Myelofibrosismentioning

confidence: 99%

“…Treatment with deferasirox in patients with MF can be associated with the onset of extra-hematological adverse events (AEs), which are primarily constituted by renal impairment: 17.7% (70), hepatic impairment: 8.9% (68), gastrointestinal symptoms: 11.1-21.4% (68,69), and skin reactions: 6.7-7.2% (68,69). Overall, a definitive discontinuation of ICT secondary to toxicity of grade ≥2 of DFX was reported in about a quarter of patients.…”

Section: Safety Profile Of Deferasirox In Myelofibrosismentioning

confidence: 99%

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From Biology to Clinical Practice: Iron Chelation Therapy With Deferasirox

et al. 2021

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Iron chelation therapy (ICT) has become a mainstay in heavily transfused hematological patients, with the aim to reduce iron overload (IOL) and prevent organ damage. This therapeutic approach is already widely used in thalassemic patients and in low-risk Myelodysplastic Syndrome (MDS) patients. More recently, ICT has been proposed for high-risk MDS, especially when an allogeneic bone marrow transplantation has been planned. Furthermore, other hematological and hereditary disorders, characterized by considerable transfusion support to manage anemia, could benefit from this therapy. Meanwhile, data accumulated on how iron toxicity could exacerbate anemia and other clinical comorbidities due to oxidative stress radical oxygen species (ROS) mediated by free iron species. Taking all into consideration, together with the availability of approved oral iron chelators, we envision a larger use of ICT in the near future. The aim of this review is to better identify those non-thalassemic patients who can benefit from ICT and give practical tips for management of this therapeutic strategy.

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Section: Impact Of Deferasirox Treatment In Reducing Ferritin and Inducing Erythroid Response In Transfusion-dependent Myelofibrosismentioning

confidence: 86%

Section: Impact Of Deferasirox Treatment In Reducing Ferritin and Inducing Erythroid Response In Transfusion-dependent Myelofibrosismentioning

confidence: 89%

Section: Impact Of Deferasirox Treatment In Reducing Ferritin and Inducing Erythroid Response In Transfusion-dependent Myelofibrosismentioning

confidence: 99%

Section: Safety Profile Of Deferasirox In Myelofibrosismentioning

confidence: 99%

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From Biology to Clinical Practice: Iron Chelation Therapy With Deferasirox

et al. 2021

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“…Currently, there are no US Food and Drug Administration (FDA) or European Medicines Agency (EMA)-approved drugs for the treatment of anemia, with or without red blood cell (RBC) transfusion dependence, and thrombocytopenia in MF, but commonly used drugs include corticosteroids, androgenic steroids, erythropoietin, and immunomodulatory drugs (such as thalidomide, lenalidomide, and pomalidomide). Furthermore, given the detrimental effects of anemia and transfusion-related iron overload, a proper iron chelation therapy is crucial and seems to be effective on outcome of disease,23 in particular in terms of hematologic improvement and survival 24–26…”

Section: Management Of Mf-related Cytopeniasmentioning

confidence: 99%

Standard care and investigational drugs in the treatment of myelofibrosis

Barraco¹,

Maffioli²,

Passamonti³

2019

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Myelofibrosis (MF) is a heterogeneous disorder characterized by splenomegaly, constitutional symptoms, ineffective hematopoiesis, and an increased risk of leukemic transformation. The ongoing research in understanding the pathophysiology of the disease has allowed for the development of targeted drugs optimizing patient management. Furthermore, disease prognostication has significantly improved. Current therapeutic interventions are only partially effective with only allogeneic stem cell transplant potentially curative. Ruxolitinib is the only approved therapy for MF by the US Food and Drug Administration. However, despite efficacy in reducing splenomegaly and controlling symptomatology, it is not associated with consistent molecular or pathologic responses. Drug discontinuation is associated with a dismal outcome. The therapeutic landscape in MF has significantly improved, and emerging drugs with different target pathways, alone or in combination with ruxolitinib, seem promising.

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Iron overload disorders

et al. 2022

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Iron overload disorders represent a variety of conditions that lead to increased total body iron stores and resultant end‐organ damage. An elevated ferritin and transferrin‐iron saturation can be commonly encountered in the evaluation of elevated liver enzymes. Confirmatory homeostatic iron regulator (HFE) genetic testing for C282Y and H63D, mutations most encountered in hereditary hemochromatosis, should be pursued in evaluation of hyperferritinemia. Magnetic resonance imaging with quantitative assessment of iron content or liver biopsy (especially if liver disease is a cause of iron overload) should be used as appropriate. A secondary cause for iron overload should be considered if HFE genetic testing is negative for the C282Y homozygous or C282Y/H63D compound heterozygous mutations. Differential diagnosis of secondary iron overload includes hematologic disorders, iatrogenic causes, or chronic liver diseases. More common hematologic disorders include thalassemia syndromes, myelodysplastic syndrome, myelofibrosis, sideroblastic anemias, sickle cell disease, or pyruvate kinase deficiency. If iron overload has been excluded, evaluation for causes of hyperferritinemia should be pursued. Causes of hyperferritinemia include chronic liver disease, malignancy, infections, kidney failure, and rheumatic conditions, such as adult‐onset Still's disease or hemophagocytic lymphohistiocytosis. In this review, we describe the diagnostic testing of patients with suspected hereditary hemochromatosis, the evaluation of patients with elevated serum ferritin levels, and signs of secondary overload and treatment options for those with secondary iron overload.

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Deferasirox in the management of iron‐overload in patients with myelofibrosis: a multicentre study from the Rete Ematologica Lombarda (IRON‐M study)

Cited by 11 publications

References 15 publications

From Biology to Clinical Practice: Iron Chelation Therapy With Deferasirox

From Biology to Clinical Practice: Iron Chelation Therapy With Deferasirox

Standard care and investigational drugs in the treatment of myelofibrosis

Iron overload disorders

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