Incidence of Hepatocellular Carcinoma in a Thalassemia Unit

Fragatou, Soso; Tsourveloudis, Ioannis; Manesis, George

doi:10.3109/03630269.2010.485071

Cited by 26 publications

(43 citation statements)

References 15 publications

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“…The direct role of HCV infection in progression to HCC among patients with thalassemia is suggested by several reports in the literature. In a recent report on HCC incidence in a Greek thalassemia unit, HCC developed in 2 patients with TM who were not iron overloaded but had HCV‐related cirrhosis that failed to respond to antiviral treatment . The results from this study suggest that HCV infection is 1 of the main culprits of HCC development in patients with TM.…”

Section: Risk Factorsmentioning

confidence: 55%

“…In the general population, the median age at HCC onset in developed countries is approximately 70 years, with a 2.4:1 male‐to‐female ratio; on the other hand, most patients with thalassemia develop HCC at age <50 years, and there is no discernible difference in incidence between men and women in this group of patients . It is worth mentioning that HCC seems to be more common in patients with TI than TM, with a male‐to‐female ratio ranging from 2:1 to 9.5:1 . One possible explanation is that patients who have TI usually have improved survival compared with those who have TM, which enables them to live long enough to develop HCC …”

Section: Incidencementioning

confidence: 97%

“…The American Association for the Study of Liver Diseases recommends surveillance of cirrhotic patients in the general population using biannual abdominal ultrasound, and it recognizes that α‐fetoprotein (AFP) measurement lacks the sensitivity and specificity required for optimal HCC detection . The inadequate sensitivity of AFP for HCC screening is also apparent in patients with thalassemia, in whom AFP levels were normal in 44% of patients in an Italian study and in 100% of those in another study by Greek investigators …”

Section: Managementmentioning

confidence: 99%

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Hepatocellular carcinoma as an emerging morbidity in the thalassemia syndromes: A comprehensive review

Moukhadder

Halawi

Cappellini

et al. 2016

Cancer

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The incidence of hepatocellular carcinoma (HCC) in patients with thalassemia is on the rise. The 2 well recognized HCC risk factors in thalassemia are iron overload and chronic viral infection with hepatitis C. The carcinogenicity of iron is related to its induction of oxidative damage, which results in genotoxicity, and to immunologic dysregulation, which attenuates cancer immune surveillance. Chronic hepatitis B and C infections lead to necroinflammation, which can prompt progression to HCC, but an independent role of hepatitis B virus in hepatic carcinogenesis among patients with thalassemia has not been demonstrated. Screening patients who have thalassemia using magnetic resonance imaging-based liver iron concentration measurement and liver ultrasound is recommended for early detection of iron overload and HCC, respectively. Prevention primarily resides in hepatitis B vaccination, donor blood screening, hepatitis treatment, and iron chelation. Although solid data is lacking on the outcomes of HCC treatment in patients with thalassemia, a personalized approach tailored to the individual patient's comorbidities remains necessary for treatment success. Treatment modalities for HCC include surgical resection, chemoembolization, and liver transplantation, among others. Multicenter studies are needed to better explore therapeutic targets that can improve the prognosis of these patients. Cancer 2017;123:751-58. © 2016 American Cancer Society.

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Section: Risk Factorsmentioning

confidence: 55%

Section: Incidencementioning

confidence: 97%

Section: Managementmentioning

confidence: 99%

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Hepatocellular carcinoma as an emerging morbidity in the thalassemia syndromes: A comprehensive review

Moukhadder

Halawi

Cappellini

et al. 2016

Cancer

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“…(Borgna‐Pignatti et al , ) Subsequently a few studies have described the appearance of HCC in thalassaemia patients. In a study from Greece, 2/57 patients with TM and 3 out of 9 with TI were found to have HCC, (Fragatou et al , ) and an Iranian study recently reported one case (Ansari et al , ). Maakaron et al () reported two TI cases and reviewed the pertinent literature, suggesting that this complication might be more frequent in TI than in TM.…”

Section: Discussionmentioning

confidence: 98%

Hepatocellular carcinoma in thalassaemia: an update of the Italian Registry

et al. 2014

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Summary The risk of developing hepatocellular carcinoma (HCC) in patients with thalassaemia is increased by transfusion‐transmitted infections and haemosiderosis. All Italian Thalassaemia Centres use an ad hoc form to report all diagnoses of HCC to the Italian Registry. Since our last report, in 2002, up to December 2012, 62 new cases were identified, 52% of whom were affected by thalassaemia major (TM) and 45% by thalassaemia intermedia (TI). Two had sickle‐thalassaemia (ST). The incidence of the tumour is increasing, possibly because of the longer survival of patients and consequent longer exposure to the noxious effects of the hepatotropic viruses and iron. Three patients were hepatitis B surface antigen‐positive, 36 patients showed evidence of past infection with hepatitis B virus (HBV). Fifty‐four patients had antibodies against hepatitis C virus (HCV), 43 of whom were HCV RNA positive. Only 4 had no evidence of exposure either to HCV or HBV. The mean liver iron concentration was 8 mg/g dry weight. Therapy included chemoembolization, thermoablation with radiofrequency and surgical excision. Three patients underwent liver transplant, 21 received palliative therapy. As of December 2012, 41 patients had died. The average survival time from HCC detection to death was 11·5 months (1·4–107·2 months). Ultrasonography is recommended every 6 months to enable early diagnosis of HCC, which is crucial to decrease mortality.

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“…Cette atteinte hépa-tique s'accompagne d'une élévation des transaminases, anomalie biologique persistante chez cette patiente (Tab. I) [11]. Il peut également exister des complications endocriniennes, notamment un diabète insulino-dépendant chez 10 à 15 % des patients.…”

Section: Commentairesunclassified

Bêta-thalassémie majeure : actualisation de la prise en charge en médecine bucco-dentaire

Millot

Woimant

Ejeil

et al. 2011

Med Buccale Chir Buccale

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Mots clés : thalassémie majeure / ostéoporose / bisphosphonates / infectionRésumé -La β-thalassémie majeure représente un syndrome génétique qui s'accompagne d'un défaut de synthèse de l'hémoglobine et entraine une anémie sévère. Malgré des progrès importants dans la prise en charge des patients, il existe toujours de nombreuses complications dues à la maladie elle-même, aux transfusions répétées, à la surcharge en fer et à la toxicité des chélateurs du fer ; elles dominent encore le pronostic vital. Toutes ces complications et/ou leurs traitements ont un impact sur la prise en charge bucco-dentaire des patients ; le cas rapporté en constitue un exemple. Tout praticien peut rencontrer dans sa pratique un patient atteint d'une β-thalassémie majeure : il est indispensable de connaitre les mesures particulières qui doivent être prises lors de l'anamnèse, de l'examen clinique, des interventions chirurgicales et de la surveillance postopératoire. Key words: thalassemia major / osteoporosis / bisphosphonates / infectionAbstract -β-thalassemia major: news repercussions of this pathology and clinical managment in oral surgery. Beta-thalassemias major are hereditary disorders resulting from defects in hemoglobin production and lead to severe anemia. Despite major advances in clinical management, several complications due to disease, lifelong transfusions, iron overload, iron chelation toxicity persist and still influence the prognosis. Those complications and/or their treatments have oral repercussions; here we present an example. Clinicians may encounter patients with beta-thalassemias in daily practice and it is essential to know the precautions that should be taken during medical history, oral examination, oral surgery and during recovery after surgery.Les ß-thalassémies sont des maladies génétiques, à transmission généralement autosomique récessive, résultant d'une insuffisance de production des chaines de ß-globine [1]. Ces hémoglobinopathies sont très répandues dans le bassin mé-diterranéen mais leur distribution tend à devenir ubiquitaire en raison du mouvement des populations. On différencie la ß-thalassémie hétérozygote ou mineure de la thalassémie homozygote ou thalassémie majeure (β-TM). Le tableau clinique de la ß-thalassémie est variable : elle peut être asymptomatique ou rapidement létale pour les formes les plus sévères. Les chaines de globine alpha en excès précipitent et leurs produits de dégradation dans les globules rouges sont à l'origine d'une érythropoïèse inefficace et d'une destruction des globules rouges dans la circulation sanguine. Dans la β-TM, la première manifestation clinique est l'apparition d'une ané-mie sévère et précoce. Cette anémie stimule la production

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Incidence of Hepatocellular Carcinoma in a Thalassemia Unit

Cited by 26 publications

References 15 publications

Hepatocellular carcinoma as an emerging morbidity in the thalassemia syndromes: A comprehensive review

Hepatocellular carcinoma as an emerging morbidity in the thalassemia syndromes: A comprehensive review

Hepatocellular carcinoma in thalassaemia: an update of the Italian Registry

Bêta-thalassémie majeure : actualisation de la prise en charge en médecine bucco-dentaire

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