Diagnosis and management of transfusion iron overload: The role of imaging

Wood, John C.

doi:10.1002/ajh.21099

Cited by 81 publications

(71 citation statements)

References 18 publications

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“…Imaging studies such as measurement of hepatic and cardiac iron are more accurate and have less variability than does serum ferritin and are becoming more available and could provide a more accurate assessment of iron overload in future studies. 18 The toxicity of iron overload is attributed to the increased generation of hydroxyl radicals by nontransferrin bound iron (NTBI) on exposure to the conditioning regimen. 19,20 In a study of 10 patients undergoing allogeneic HCT, transferrin saturation rose and NTBI rapidly appeared after initiation of the conditioning regimen, peaked as early as day 4 and was detectable for an average of 14 days.…”

Section: Discussionmentioning

confidence: 99%

Iron overload adversely affects outcome of allogeneic hematopoietic cell transplantation

Pullarkat

Blanchard

Tegtmeier

et al. 2008

Bone Marrow Transplant

191

176

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Iron overload is common in patients undergoing allogeneic hematopoietic cell transplantation (HCT) for hematologic disorders. Serum ferritin, a marker of tissue iron overload, was measured immediately before transplant in adult patients undergoing myeloablative HCT from matched sibling or unrelated donors. The effect of elevated pretransplant ferritin (defined as ferritin X1000 ng/ml) on day 100 mortality, overall survival, acute GVHD and infectious complications was assessed. Data on 190 patients were analyzed. In univariate analysis, the highferritin group had increased day 100 mortality (20 vs 9%, P ¼ 0.038), decreased overall survival (log-rank test: P-value ¼ 0.004), increased acute GVHD/death (63 vs 43%, P ¼ 0.009) and increased incidence of blood stream infections (BSIs)/death (60 vs 44%, P ¼ 0.042). In a multivariate analysis, high ferritin was associated with increased risk of death (Cox model: hazard ratio ¼ 2.28, P ¼ 0.004), increased day 100 mortality (generalized linear model (GLM) odds ratio ¼ 3.82, P ¼ 0.013), increased incidence of acute GVHD/death (GLM odds ratio ¼ 3.11, P ¼ 0.001) and increased risk of BSI/death (GLM odds ratio ¼ 1.99, P ¼ 0.032). The results remained similar when serum ferritin was considered a continuous variable. Elevated serum ferritin adversely impacts on overall survival and increases the likelihood of acute GVHD and BSI after allogeneic HCT.

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Section: Discussionmentioning

confidence: 99%

Iron overload adversely affects outcome of allogeneic hematopoietic cell transplantation

Pullarkat

Blanchard

Tegtmeier

et al. 2008

Bone Marrow Transplant

191

176

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“…In transfusion dependent patients, the iron in the liver represents the total body iron load, as it stores 70%-80% of the iron [20]. Patients with high levels of Liver iron concentration (LIC) are at increased risk of developing cardiac iron compared with patients with low LIC [21].…”

Section: Assessment Of Iron Overloadmentioning

confidence: 99%

Dental considerations in Thalassemic patients

Madhok¹,

Madhok²

2014

IOSRJDMS

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“…However, although the use of CMR is spreading [13], its availability is so far limited, constituting worldwide for many centers, where thalassemia is common, one of the main crucial issue for the right management of patients [14]. Instead, worldwide availability of echocardiography is surely greater.…”

mentioning

confidence: 99%

Long‐term use of deferiprone significantly enhances left‐ventricular ejection function in thalassemia major patients

et al. 2012

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A multicenter randomized open-label long-term sequential deferiprone-deferoxamine (DFP-DFO) versus DFP alone trial (sequential DFP-DFO) performed in patients with thalassemia major (TM) was retrospectively reanalyzed to assess the variation in the left ventricular ejection fraction (LVEF) [1].Serial observations of LVEF over 3 years, in the same patient, were retrospectively assessed in 99 patients with TM during the sequential DFP-DFO multicenter randomized open-label trial [1]. A generalized estimating equation (GEE) model was used to demonstrate changes in mean LVEF over time [2].The regression coefficient of treatment suggested that the DFP-alone group showed a statistically significant increase in mean LVEF over time (coefficient 0.97, 95% CI (0.51; 1.44), P-value <0.0001).These findings suggest that long-term treatment with DFP-alone can significantly enhance LVEF over time. These findings agree with a survival analysis reporting a substantial decline in cardiac deaths during recent years, related to the switching of high-risk patients from DFO to chelation regimens that include the oral chelator DFP [3][4][5].Oral chelation treatment has improved greatly adherence and management of patients with TM [6,7].The improvement of the LVEF after 1-year DFP treatment has been reported [8][9][10][11].However, the effects of DFP on LVEF after long-term treatment have not been fully investigated.This letter reports a retrospective survey performed on patients with TM, previously enrolled in a long-term randomized open-label trial carrying ahead in Italy on the behalf of the Italian Society for the Study of Thalassaemia and Haemoglobinopathies (SoSTE) [1]. Ninety-nine out of 213 patients enrolled in the sequential DFP-DFO trial underwent longterm echocardiographic study of LVEF measured at baseline and every 12 months over three consecutive years (Fig. 1). Among these, 39 and 60 received sequential DFP (75 mg/kg for 4 days/week)-DFO (50 mg/kg for 3 days/week) or DFP-alone (75 mg/kg for 7 days/week) treatment, respectively (Table I).The hematological and clinical findings at enrollment are shown in Table I. No differences were observed at baseline between the two randomized groups. Particularly, the main findings of body iron overloading, expressed as serum ferritin at baseline, liver iron concentration (LIC), baseline LVEF <55%, and total number of blood transfusions were not statistically significantly different (Table I). Moreover, although baseline LVEF appears unlike between the two groups ( Fig. 1), this was not statistically significantly different (P-value 5 0.10, Table I).The DFP-alone group showed statistically significant increase over time in mean LVEF in comparison with sequential DFO-FP treatment (coefficient 0.97, 95% CI (0.51; 1.44), P-value <0.0001).Furthermore, the regression coefficient of treatment suggested that there was a statistically significant difference in mean LVEF between the two treated groups favoring the sequential group (coefficient 2.36, 95% CI (0.02; 4.71), P-value 5 0.047, but this ...

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Diagnosis and management of transfusion iron overload: The role of imaging

Cited by 81 publications

References 18 publications

Iron overload adversely affects outcome of allogeneic hematopoietic cell transplantation

Iron overload adversely affects outcome of allogeneic hematopoietic cell transplantation

Dental considerations in Thalassemic patients

Long‐term use of deferiprone significantly enhances left‐ventricular ejection function in thalassemia major patients

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