Iron overload in lower international prognostic scoring system risk patients with myelodysplastic syndrome receiving red blood cell transfusions: Relation to infections and possible benefit of iron chelation therapy

2018 ◽  
Vol 67 ◽  
pp. 75-81 ◽  
Author(s):  
Colleen A.C. Wong ◽  
Shannon A.Y. Wong ◽  
Heather A. Leitch
Keyword(s):  
Blood Cell ◽  
Myelodysplastic Syndrome ◽  
Iron Overload ◽  
Chelation Therapy ◽  
Iron Chelation ◽  
International Prognostic Scoring System ◽  
Iron Chelation Therapy ◽  
System Risk ◽  
Risk Patients ◽  
Red Blood Cell Transfusions

scholarly journals Durable Red Blood Cell Transfusion Independence in a Patient with an MDS/MPN Overlap Syndrome Following Discontinuation of Iron Chelation Therapy

2015 ◽  
Vol 2015 ◽  
pp. 1-7 ◽  
Author(s):  
Harpreet Kochhar ◽  
Chantal S. Leger ◽  
Heather A. Leitch
Keyword(s):  
Blood Cell ◽  
Iron Overload ◽  
Red Blood Cell ◽  
Chelation Therapy ◽  
Iron Chelation ◽  
Red Blood Cell Transfusion ◽  
Favorable Effect ◽  
Iron Chelation Therapy ◽  
Transfusion Independence ◽  
Rbc Transfusion

Background. Hematologic improvement (HI) occurs in some patients with acquired anemias and transfusional iron overload receiving iron chelation therapy (ICT) but there is little information on transfusion status after stopping chelation.Case Report. A patient with low IPSS risk RARS-T evolved to myelofibrosis developed a regular red blood cell (RBC) transfusion requirement. There was no response to a six-month course of study medication or to erythropoietin for three months. At 27 months of transfusion dependence, she started deferasirox and within 6 weeks became RBC transfusion independent, with the hemoglobin normalizing by 10 weeks of chelation. After 12 months of chelation, deferasirox was stopped; she remains RBC transfusion independent with a normal hemoglobin 17 months later. We report the patient’s course in detail and review the literature on HI with chelation.Discussion. There are reports of transfusion independence with ICT, but that transfusion independence may be sustained long term after stopping chelation deserves emphasis. This observation suggests that reduction of iron overload may have a lasting favorable effect on bone marrow failure in at least some patients with acquired anemias.

Quality of Life and Economic Impact of Red Blood Cell (RBC) Transfusions on Patients with Myelodysplastic Syndromes (MDS).

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 4716-4716 ◽  
Author(s):  
S. Brechignac ◽  
E. Hellstrom-Lindberg ◽  
D. T. Bowen ◽  
T. M. DeWitte ◽  
M. Cazzola ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Blood Cell ◽  
Red Blood Cell ◽  
Chelation Therapy ◽  
Subcutaneous Administration ◽  
Iron Chelation ◽  
International Prognostic Scoring System ◽  
Blood Collection ◽  
Iron Chelation Therapy

Abstract Background: Supportive care with blood product transfusions is the primary management strategy for the majority of patients with MDS. Approximately 80% of MDS patients are anemic at the time of presentation and more than 40% require regular RBC transfusions at some stage of disease, while platelet transfusions are less often required. Methods: In an effort to systematically study quality of life and economic cost associated with transfusion dependency (especially RBC transfusions), The MDS Foundation has disseminated a practices and treatment survey to its Centers of Excellence and is also accumulating transfusion data. Retrospective and prospective data collected include hematologic parameters defining transfusion need; percentage (%) of MDS patients requiring transfusion; % of transfusion-dependent MDS patients by subtype and International Prognostic Scoring System (IPSS) risk group; per patient frequency of transfusions; % of patients requiring iron chelation therapy. Results: A total of 30 Centers have replied to the survey to date, and responses reveal that a substantial proportion of MDS patients receive multiple RBC transfusions with most of these patients needing chelation therapy with desferoxamine (generally subcutaneous administration, 4-times weekly): Table 1. In addition, detailed data are available from 4 European Centers that have provided transfusion records from randomly selected multiply-transfused MDS patients: 38 patients (median age: 73) received a median of 42 transfusions over the last 24 months (range: 11–207). The average per transfusion costs calculated from estimates provided by the 4 European centers is 436 euros or $ 526 ($1 US dollar = 0.83 euros), where the per transfusion cost includes 2 filtered red blood cell units, blood collection, administrative costs, and staff time, resulting in a median per patient cost over the last 24 months of 11,118 euros (range: 5668–21,800 euros). This does not include the cost of chelation therapy (300 euros/month for desferioxamine SC) and indirect costs (e.g., time spent at transfusion facility, travel time for patient to facility, travel and wait time for private caretaker or family member). Conclusion: Preliminary data analysis from the ongoing retrospective study suggests that the transfusion burden to MDS patients and to society, in terms of quality of life and cost, is much greater than generally appreciated. Updated data of this study will be presented. Table 1: RBC Transfusion-dependent MDS patients Mean % IPSS low risk 39 IPSS intermediate-1 risk 50 IPSS intermediate-2 risk 63 IPSS high risk 79 Iron chelation therapy 28

Should Patients with Myelodysplastic Syndromes Undergo Iron Chelation Therapy? New Data Answering the Question

2009 ◽  
Vol 02 ◽  
pp. 60
Author(s):  
Stuart L Goldberg ◽  
Keyword(s):  
Iron Overload ◽  
Myelodysplastic Syndromes ◽  
Scoring System ◽  
Chelation Therapy ◽  
Iron Chelation ◽  
World Health ◽  
International Prognostic Scoring System ◽  
Iron Chelation Therapy ◽  
Older Individuals ◽  
Prospective Trials

Although it is well established that transfusional iron overload leads to organ impairment and shortened survival among children with thalassemia and sickle cell anemia, the role of transfusional iron overload and its treatment among older individuals with the myelodysplastic syndromes (MDS) remains unclear. Recent reviews have noted that MDS patients requiring frequent red blood cell transfusions experience higher rates of cardiac, hepatic, endocrine, and other organ damage in patterns similar to pediatric transfusional hemochromatosis, with increased rates of transformation to leukemia and decreased survival. Through the use of prognostic scoring systems such as the International Prognostic Scoring System (IPSS) and the World Health Organization (WHO) classification-based prognostic scoring system (WPSS), MDS patients with projected survivals long enough to warrant concern about the toxicities of iron overload can be identified and iron chelation therapies can be considered. The results of two large prospective trials among transfusion-dependent MDS patients, US03 and EPIC, have demonstrated that the oral iron chelation agent deferasirox can successfully reduce iron content and has an acceptable safety profile in this elderly population. Furthermore, retrospective trials have suggested that iron chelation therapy may prolong survival in MDS, and prospective trials are being planned. This article will highlight some of these issues.

scholarly journals Iron Chelation Therapy with Deferasirox Results in Improvement of Liver Enzyme Level in Patients with Iron Overload-Associated Liver Dysfunction

2010 ◽  
Vol 2010 ◽  
pp. 1-3 ◽  
Author(s):  
Yasuo Miura ◽  
Yusuke Matsui ◽  
Hitomi Kaneko ◽  
Mitsumasa Watanabe ◽  
Mitsuru Tsudo
Keyword(s):  
Iron Overload ◽  
Liver Dysfunction ◽  
Chelation Therapy ◽  
Enzyme Level ◽  
Elevated Serum ◽  
Iron Chelation ◽  
Iron Chelator ◽  
Similar Efficacy ◽  
Iron Chelation Therapy ◽  
Red Blood Cell Transfusions

Iron chelation therapy (ICT) has been applied for the patients with iron overload-associated liver dysfunction since it is one of the causes of death in patients with intractable hematological diseases requiring multiple red blood cell transfusions. Recently, deferasirox (DSX), a novel, once-daily oral iron chelator, was demonstrated to have similar efficacy to the conventional continuous infusion of deferoxamine on a decrease in serum ferritin (SF) level in heavily transfused patients. We show three cases of transfusion-mediated iron-overloaded patients with an elevated serum alanine aminotransaminase (ALT). All three patients who received the ICT with DSX showed a decrease in ALT level in association with a decrease in SF level. It is suggested that DSX therapy could be considered to expect the improvement of liver damage for iron-overloaded patients with an abnormal ALT level.

Evolution of iron overload in patients with low-risk myelodysplastic syndrome: iron chelation therapy and organ complications

2014 ◽  
Vol 94 (5) ◽  
pp. 779-787 ◽  
Author(s):  
Ángel F. Remacha ◽  
◽  
Beatriz Arrizabalaga ◽  
Ana Villegas ◽  
María Soledad Durán ◽  
...  
Keyword(s):  
Myelodysplastic Syndrome ◽  
Iron Overload ◽  
Chelation Therapy ◽  
Iron Chelation ◽  
Low Risk ◽  
Iron Chelation Therapy ◽  
Organ Complications

The IRON2 Study. A Retrospective Observational Study to Describe the Evolution of Iron Overload in Patients with Low-Risk Myelodysplastic Syndrome

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 1723-1723 ◽  
Author(s):  
Angel Remacha ◽  
Beatriz Arrizabalaga ◽  
Ana Villegas ◽  
Maria Soledad Duran ◽  
Lourdes Hermosin ◽  
...  
Keyword(s):  
Observational Study ◽  
Myelodysplastic Syndrome ◽  
Iron Overload ◽  
Chelation Therapy ◽  
Iron Chelation ◽  
Median Number ◽  
Low Risk ◽  
Cardiac Complications ◽  
Iron Chelation Therapy ◽  
Free Survival

Abstract Abstract 1723 Introduction and Objective: A large number of patients with low-risk myelodysplastic syndrome (MDS) need blood transfusions, being iron overload an inevitable consequence that may cause hepatic fibrosis and cirrhosis, diabetes or myocardiopathy unless it is not treated. The main objective of this study is to describe the evolution of iron overload in transfusion-dependent patients with low-risk MDS. Secondary objectives include the evaluation of the impact of iron chelation therapy on Event Free Survival (EFS) (including infections, arthropathy, diabetes mellitus, hepatic and cardiac complications), Overall Survival (OS) and Leukemia Free Survival (LFS). Methods and Patients: Retrospective observational study carried out in Haematology Departments of 47 Spanish hospitals between March 2010 and March 2011. Transfusion-dependent patients with low/intermediate-1 IPSS risk who had received ≥10 red blood cell (RBC) transfusion during at least 12 months previous to study entry were eligible. Results: A total of 263 patients were evaluated [age at diagnosis, 74 ± 10.5 years]. IPSS classification was available in 82.9% included patients (lack of cytogenetics in 37 patients), classified as low risk (86%) and int-1 risk (14%). At diagnosis, 25.5% of patients had serum ferritin (SF) levels >500ng/ml, 8.7% SF>1,000ng/ml, and 22% TSI>50%. The median number of RBC transfusions per month was 2.35 RBC/month; during the course of the disease, 82.4% of patients reached SF levels >1,000ng/ml. Cardiac complications worsened/appeared in 24.4% of patients, having received a median RBC transfusions of 22 (7–92), and showing a median SF levels of 1,365 (735–3,025) ng/ml. One-hundred forty-seven (55.9%) patients started iron chelation therapy (85.5% with deferasirox) with a median number of RBC transfused of 23 (14–38) and a median SF levels of 1,570 (1,231.5–2,195) ng/ml. 71% of patients with SF>1,000ng/ml were on iron chelation therapy. Table 1 shows the results of univariate analysis of EFS, OS and LFS. As part of this study, we are currently performing a multivariate analysis whose results will be presented in forthcoming congress. Conclusions: The results of this study show that a high percentage of transfusion-dependent patients with low-risk MDS (82.4%) reach SF levels >1,000ng/ml during the course of their disease. Patients on iron chelation therapy show a higher cardiac EFS, OS and LFS compared with those not treated, being deferasirox the most frequently used chelating agent in clinical practice. Disclosures: Sanz: Novartis Farmaceutica S.A.: Employment.

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