Brain positron emission tomography in splenectomized adults with β-thalassemia intermedia: uncovering yet another covert abnormality

Musallam, Khaled M.; Nasreddine, Wassim; Beydoun, Ahmad; Hourani, Roula; Hankir, Ahmed; Koussa, Suzanne; Haidar, Mohamad; Taher, Ali T.

doi:10.1007/s00277-011-1291-3

Cited by 29 publications

(20 citation statements)

References 38 publications

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“…Similar studies from pediatric patients failed to document a high rate of silent strokes [52]. The aforementioned hypercoagulable state in TI alongside iron overload with subsequent cerebrovascular damage [53,54] could be involved in the pathophysiology of silent cerebral infarction. Further study of the ideal prevention strategies of these covert lesions is warranted, especially whether they could be associated with neurocognitive disabilities [55].…”

Section: Thrombosis In Thalassemiamentioning

confidence: 90%

Hypercoagulability in β-thalassemia: a status quo

Cappellini¹,

Poggiali

Taher

et al. 2012

Expert Review of Hematology

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Section: Thrombosis In Thalassemiamentioning

confidence: 90%

Hypercoagulability in β-thalassemia: a status quo

Cappellini¹,

Poggiali

Taher

et al. 2012

Expert Review of Hematology

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“…Recent studies have also documented a high prevalence of large cerebral vessel disease (magnetic resonance angiography) and decreased neuronal function (positron emission tomography-computed tomography) primarily in the temporal and parietal lobes in similar patient cohorts. [66][67][68] A significant association between the occurrence of these abnormalities and elevated iron overload indices was noted. 67,68 In the general population, and in patients with sickle cell disease, these silent cerebrovascular abnormalities are associated with subsequent risk of overt stroke and neurocognitive decline, further highlighting the importance of these findings.…”

Section: 57mentioning

confidence: 91%

“…[66][67][68] A significant association between the occurrence of these abnormalities and elevated iron overload indices was noted. 67,68 In the general population, and in patients with sickle cell disease, these silent cerebrovascular abnormalities are associated with subsequent risk of overt stroke and neurocognitive decline, further highlighting the importance of these findings. 66 Another vascular complication of NTDT (primarily β-thalassemia intermedia and hemoglobin E/β-thalassemia) that was found to occur at a relatively high frequency compared to patients with β-thalassemia major is pulmonary hypertension.…”

Section: 57mentioning

confidence: 91%

Non-transfusion-dependent thalassemias

Musallam

Rivella

Vichinsky³

et al. 2013

Haematologica

249

268

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“…Moreover, it was apparent that elevated LIC was associated with a steeper increase in the rate of age-related vascular morbidity and earlier onset of endocrine and bone disease compared with patients with low LIC (20). Recent studies have also documented a high prevalence of silent brain infarction, large cerebral vessel disease, and decreased neuronal function primarily in the temporal and parietal lobes in splenectomized adults with b-thalassemia intermedia (33)(34)(35). There was a significant association between the occurrence of large-vessel cerebrovascular disease and high NTBI levels (34), and decreased neuronal function was observed more frequently in patients with LIC >15 mg Fe/g dw (33).…”

Section: Clinical Consequencesmentioning

confidence: 99%

Iron Overload in Non-Transfusion-Dependent Thalassemia

Musallam

2013

Thalassemia Reports

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Iron overload due to increased intestinal iron absorption remains a concern in patients with non-transfusion-dependent thalassemia (NTDT). A dynamic regulation between ineffective erythropiesis and iron metabolism in these disorders has been recently elucidated. Although the rate of iron loading in NTDT is slower than that observed in regularly transfused patients, the process is cumulative and patients may reach considerably high liver iron concentration levels. The clinical consequences of iron overload in patients with NTDT are various and include hepatic disease, endocrinopathy, bone disease, and vascular outcomes; while cardiac siderosis is less frequently observed. Although serum ferritin levels correlate with LIC in NTDT, they underestimate iron load when compared with transfusion-dependent patients with equivalent LIC. Therefore, direct measurement of LIC is recommended to identify patients at risk and guide iron chelation decisions. IntroductionTransfusion-dependence is an essential factor in characterizing the various phenotypes and their severity within the thalassemia syndromes. For instance, a diagnosis of b-thalassemia major entails lifelong transfusion requirement for normal growth and development, and more importantly, survival. The main concern with transfusiondependence is secondary iron overload, which if left untreated, leads to target-organ toxicity and death (1). However, considerable advances have been attained, especially in the last decade, in iron overload assessment and management strategies for transfusion-dependent patients; which translated into improved patient outcomes (2-3). Nontransfusion-dependent thalassemia (NTDT) is a term used to label patients who do not require such regular transfusions for survival and encompasses several entities, the most prevalent of which are a-thalassemia (mainly HbH disease), hemoglobin E/b-thalassemia, and bthalassemia intermedia (4). Despite their transfusion-independence, patients with NTDT are still at risk of iron overload (5). Only recently, data on the mechanisms, consequences, and assessment of iron overload in NTDT started to emerge; and these will be herein reviewed. PathophysiologyPatients with NTDT may still require occasional blood transfusions; for example, in the case of poor growth or development during childhood, during infection or pregnancy, prior to surgery, or for the management of specific complications where the benefit of transfusion therapy has been established (6). Blood transfusions in NTDT patients, however, contribute much less to the total iron burden than they do in transfusion-dependent patients, and the main source of iron remains primary loading, similar to patients with hereditary hemochromatosis. The combination of ineffective erythropoiesis, anemia, and hypoxia leads to a compensatory increase in serum levels of erythropoietin, as well as a decrease in serum levels of hepcidin, which control the concentration of ferroportin on the intestinal epithelium (7). Proposed regulators of hepcidin production incl...

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Brain positron emission tomography in splenectomized adults with β-thalassemia intermedia: uncovering yet another covert abnormality

Cited by 29 publications

References 38 publications

Hypercoagulability in β-thalassemia: a status quo

Hypercoagulability in β-thalassemia: a status quo

Non-transfusion-dependent thalassemias

Iron Overload in Non-Transfusion-Dependent Thalassemia

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