Management of Transfusion-Related Iron Overload in Patients With Myelodysplastic Syndromes

Shah, Jay; Kurtin, Sandra; Arnold, Louise; Lindroos-Kolqvist, Petra; Tinsley, Sara

doi:10.1188/12.cjon.s1.37-46

Cited by 13 publications

(29 citation statements)

References 56 publications

(98 reference statements)

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“…In the third article, Shah, Kurtin, Arnold, Lindroos-Kolqvist, and Tinsley (2012) provide an update on iron chelation therapy for treatment of transfusion-associated iron overload. However, a small number of patients achieve a prolonged response to allogeneic bone marrow transplantation.…”

Section: Support Strategiesmentioning

confidence: 99%

“…Shah et al (2012) also provide a review of the physiology of transfusion-related iron overload, strategies for identifying and monitoring at-risk patients, and guidelines for the safe administration of iron chelation therapies. Shah et al (2012) also provide a review of the physiology of transfusion-related iron overload, strategies for identifying and monitoring at-risk patients, and guidelines for the safe administration of iron chelation therapies.…”

Section: Support Strategiesmentioning

confidence: 99%

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Myelodysplastic Syndromes

Kurtin¹

2012

Clinical Journal of Oncology Nursing

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Myelodysplastic syndromes (MDS) represent a group of clonal myeloid malignancies with variability in clinical presentation and disease trajectory, as well as prognosis and treatment recommendations (Kurtin & Demakos, 2010). MDS is considered to be a rare disease that is most common in adults older than age 70. The disease is characterized by ineffective hematopoiesis, progressive bone marrow failure, and a variable risk of leukemic transformation thought to result from complex interactions between the malignant clone and the bone marrow microenvironment (Kurtin, 2011). This supplement is intended to provide the oncology clinician with an overview of MDS and provide tools for the clinical management and support of patients with MDS.

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Section: Support Strategiesmentioning

confidence: 99%

Section: Support Strategiesmentioning

confidence: 99%

Myelodysplastic Syndromes

Kurtin¹

2012

Clinical Journal of Oncology Nursing

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“…Patients with transfusion‐dependent β‐thalassemia and patients with sickle cell disease (SCD) undergo regular blood transfusions to prevent complications of disease, such as pain, acute chest syndrome, aplastic crisis, stroke, multiorgan failure, and ultimately death . However, each unit of packed red blood cells contains approximately 250 mg of iron, and after about 20 transfusions, the body accumulates excess iron due to saturation of transferrin . Breakdown of mature red blood cells over time, in the absence of erythropoiesis, results in an accumulation of non‐transferrin‐bound iron (NTBI) once the transferrin‐binding capacity has been exceeded .…”

Section: Introductionmentioning

confidence: 99%

“…However, each unit of packed red blood cells contains approximately 250 mg of iron, and after about 20 transfusions, the body accumulates excess iron due to saturation of transferrin . Breakdown of mature red blood cells over time, in the absence of erythropoiesis, results in an accumulation of non‐transferrin‐bound iron (NTBI) once the transferrin‐binding capacity has been exceeded . In turn, the presence of NTBI results in iron deposits in the cells of various organs (including liver, heart, pancreas, endocrine glands, and joints) and the formation of reactive oxygen species .…”

Section: Introductionmentioning

confidence: 99%

The effect of iron chelation therapy on overall survival in sickle cell disease and β‐thalassemia: A systematic review

et al. 2018

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Red blood cell transfusions have become standard of care for the prevention of life-threatening anemia in patients with β-thalassemia and sickle cell disease (SCD). However, frequent transfusions can lead to accumulation of iron that can result in liver cirrhosis, diabetes mellitus, arthritis, arrhythmias, cardiomyopathy, heart failure, and hypogonadotropic hypogonadism. Iron chelation therapy has been shown to reduce serum ferritin levels and liver iron content, but limitations of trial design have prevented any demonstration of improved survival. The objective of this systematic review was to investigate the impact of iron chelation therapy on overall and event-free survival in patients with β-thalassemia and SCD. Eighteen articles discussing survival in β-thalassemia and 3 in SCD were identified. Overall iron chelation therapy resulted in better overall survival, especially if it is instituted early and compliance is maintained. Comparative studies did not show any significant differences between available iron chelation agents, although there is evidence that deferiprone is better tolerated than deferoxamine and that compliance is more readily maintained with the newer oral drugs, deferiprone and deferasirox. Iron chelation therapy, particularly the second-generation oral agents, appears to be associated with improved overall and event-free survival in transfusion-dependent patients with β-thalassemia and patients with SCD.

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“…yelodysplastic syndromes (MDS) encompass a heterogeneous group of hematologic malignancies that are characterized by ineffective hematopoiesis and risk for progression to acute myeloid leukemia (AML; Mitchell, Gore, & Zeidan, 2013;Petrou et al, 2015;Shah, Kurtin, Arnold, Lindroos-Kolqvist, & Tinsley, 2012). The majority of patients with MDS (approximately 80%) are anemic, and a large percentage of them will require red blood cell (RBC) transfusional support during their disease course (Shenoy, Vallumsetla, Rachmilewitz, Verma, Ginzburg, 2014;Temraz, Santini, Musallam, & Taher, 2014).…”

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confidence: 99%

Iron Overload in Myelodysplastic Syndromes: Pathophysiology, Consequences, Diagnosis, and Treatment

Lyle¹,

Hirose²

2018

JADPRO

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Myelodysplastic syndromes (MDS) are a heterogeneous group of hematologic neoplasms varying in severity affecting one or more lines of hematopoiesis. Ineffective erythropoiesis results in dysregulation of iron metabolism. Most MDS patients have anemia, and some require regular red blood cell transfusions. These transfusions, in addition to factors of the disease itself, can result in iron overload (IO). Retrospective analyses suggest that MDS patients with IO have reduced overall survival and poorer outcomes following allogeneic stem cell transplant vs. those without IO. Iron chelation therapy (ICT; deferoxamine, deferasirox, or deferiprone) has been used to alleviate IO in other transfusiondependent hematologic conditions (e.g., thalassemia), but its role in MDS has not been firmly established. A growing body of evidence suggests that ICT in MDS patients is an effective means for reducing transfusional IO and may significantly improve outcomes such as survival. The orally administered iron chelator deferasirox has been widely studied in MDS, and available studies have shown it to be generally well tolerated and effective in reducing IO in this population. The pathophysiology and clinical consequences of IO in MDS, as well as current methods for diagnosing and treating IO in these patients, are discussed.

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Management of Transfusion-Related Iron Overload in Patients With Myelodysplastic Syndromes

Cited by 13 publications

References 56 publications

Myelodysplastic Syndromes

Myelodysplastic Syndromes

The effect of iron chelation therapy on overall survival in sickle cell disease and β‐thalassemia: A systematic review

Iron Overload in Myelodysplastic Syndromes: Pathophysiology, Consequences, Diagnosis, and Treatment

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