Improved survival in red blood cell transfusion dependent patients with primary myelofibrosis (PMF) receiving iron chelation therapy

Leitch, Heather A.; Chase, Jocelyn; Goodman, Trisha A.; Ezzat, Hatoon; Rollins, M. D.; Wong, Danielle; Badawi, Maha; Leger, Chantal S.; Ramadan, Khaled M. A.; Barnett, Michael J.; Foltz, Lynda; Vickars, Linda M.

doi:10.1002/hon.902

Cited by 31 publications

(25 citation statements)

References 61 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In a recent retrospective review, ICT was associated with superior survival in these patients [5]. Furthermore, lower transfusion requirement predicts favorable outcome in HSCT [24].…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Loss of Transfusion Dependency Following Deferasirox Treatment of Iron Overload in a Woman with Myelofibrosis and Spherocytosis - a Case Report

Tesch¹,

Ihling²

2013

Oncol Res Treat

View full text Add to dashboard Cite

Background: Many patients with primary myelofibrosis (PMF), a myeloproliferative neoplasm, become transfusion dependent due to anemia. In these patients, transfusion dependency is associated with shortened survival. Case Report: We report on a 67-year-old woman who was diagnosed with PMF. Cytogenetic testing revealed a karyotype 46,XX, del(20)(q11.2), consistent with a myeloproliferative disorder. Although offered to her because of severe anemia and leukocytosis, the patient did not consent to allogeneic hematopoietic stem cell transplantation. After having received a cumulative number of more than 70 packed red blood cell transfusions, iron chelation therapy with deferiprone was initiated to treat iron overload and switched to deferasirox after 3 months. Within 2 months of deferasirox treatment, serum ferritin concentrations were significantly reduced, the patient lost transfusion dependency for 17 months, and normal hemoglobin concentrations were recovered. Conclusion: Iron chelation treatment with deferasirox resulted in an improvement of hematologic parameters and loss of transfusion dependency.

show abstract

“…In a recent retrospective review, ICT was associated with superior survival in these patients [5]. Furthermore, lower transfusion requirement predicts favorable outcome in HSCT [24].…”

Section: Discussionmentioning

confidence: 99%

“…Currently more targeted and effective therapies are emerging. Patients with PMF frequently develop anemia, become transfusion dependent, and develop iron overload [5]. Anemia is considered an important prognostic factor in both the International Prognostic Scoring System (IPSS) [6] and the dynamic IPSS (DIPSS) [7].…”

Section: Introductionmentioning

confidence: 99%

Loss of Transfusion Dependency Following Deferasirox Treatment of Iron Overload in a Woman with Myelofibrosis and Spherocytosis - a Case Report

Tesch¹,

Ihling²

2013

Oncol Res Treat

View full text Add to dashboard Cite

show abstract

“…Advanced disease was associated with poorer survival post-HSCT (Kerbauy et al, 2007;Kröger et al, 2009a;Ciurea et al, 2010;Scott et al, 2010;Samuelson et al, 2011) while the JAK2 V617F mutation (Kröger et al, 2009b) or antecedent PV/ET may result in superior survival (Kerbauy et al, 2007). Transfusion dependency characterizes aggressive disease, with worse HSCT outcome, although iron chelation may ameliorate Leitch et al, 2010;Elena et al, 2011).…”

Section: Reduced Intensity Hsctmentioning

confidence: 99%

Guideline for the diagnosis and management of myelofibrosis

et al. 2012

View full text Add to dashboard Cite

SummaryThe guideline group regarding the diagnosis and management of myelofibrosis was selected to be representative of UK-based medical experts, together with a contribution from a single expert from the USA. MEDLINE and EMBASE were searched systematically for publications in English from 1966 until August 2011 using a variety of key words. The writing group produced the draft guideline, which was subsequently revised by consensus of the members of the General Haematology and Haemato-oncology Task Forces of the British Committee for Standards in Haematology (BCSH). The guideline was then reviewed by a sounding board of UK haematologists, the BCSH and the British Society for Haematology Committee and comments incorporated where appropriate. The criteria used to state levels and grades of evidence are as outlined in the Procedure for Guidelines commissioned by the BCSH; the 'GRADE' system was used to score strength and quality of evidence. The objective of this guideline is to provide healthcare professionals with clear guidance on the investigation and management of primary myelofibrosis, as well as post-polycythaemic myelofibrosis (post-PV MF) and post-thrombocythemic myelofibrosis (post-ET MF) in both adult and paediatric patients.

show abstract

“…Iron homeostasis can be reasonably predicted to be disturbed in this population, given the high prevalence of severe anemia, a surrogate for ineffective erythropoiesis; 38% of patients have a hemoglobin level of <10 g/dL and 24% have required red blood cell (RBC) transfusions at initial diagnosis, with increasing prevalence of anemia manifest during the disease course [1]. Additional inputs into disturbed iron homeostasis in PMF include dysregulated circulating inflammatory cytokine expression [2,3] and iron overload [4,5]; the latter is associated chiefly with increased infusional iron in the form of RBC transfusions, but also possibly abnormal gut iron absorption.…”

Section: Introductionmentioning

confidence: 99%

Associations and prognostic interactions between circulating levels of hepcidin, ferritin and inflammatory cytokines in primary myelofibrosis

et al. 2013

View full text Add to dashboard Cite

Iron homeostasis is dysregulated in primary myelofibrosis (PMF), given the high prevalence of anemia, need for red blood cell (RBC) transfusions, and disease-associated inflammatory state. We measured plasma hepcidin levels in 203 consecutive PMF patients at the time of first referral; hepcidin levels were significantly higher as compared to healthy controls (P < 0.0001), and were correlated with hemoglobin of <10 g/dL, RBC transfusion requirement, serum ferritin of >500 mg/L, higher dynamic international prognostic scoring system (DIPSS)-plus risk category, the presence of circulating blasts, age of >65 years, and leukocyte count of <4 3 10 9 /L. Increased hepcidin levels predicted for inferior survival independent of six out of the eight DIPSS-plus prognostic parameters (hazard ratio [HR] 5 1.8; P 5 0.02), but not when RBC transfusion requirement, hemoglobin of <10 g/dL, or increased serum ferritin were included in the Cox model. Multivariable analysis that considered the four overlapping prognostic variables revealed that increased hepcidin (HR 5 1.9; P 5 0.03) and increased ferritin (HR 5 2.3; P 5 0.04), but not hemoglobin of <10 g/dL or RBC transfusion requirement, independently retained their significance for predicting survival. Accordingly, increased levels of both hepcidin and serum ferritin (seen in 29% of patients) predicted inferior survival independent of DIPSS-plus or increased inflammatory cytokine levels (HR 5 2.4; P 5 0.002), and could be considered in future prognostic models for PMF. Am. J.

show abstract

Improved survival in red blood cell transfusion dependent patients with primary myelofibrosis (PMF) receiving iron chelation therapy

Cited by 31 publications

References 61 publications

Loss of Transfusion Dependency Following Deferasirox Treatment of Iron Overload in a Woman with Myelofibrosis and Spherocytosis - a Case Report

Loss of Transfusion Dependency Following Deferasirox Treatment of Iron Overload in a Woman with Myelofibrosis and Spherocytosis - a Case Report

Guideline for the diagnosis and management of myelofibrosis

Associations and prognostic interactions between circulating levels of hepcidin, ferritin and inflammatory cytokines in primary myelofibrosis

Contact Info

Product

Resources

About