Clinical and histological evaluation of liver disease in patients with transfusion-dependent beta-thalassemia: A multicenter study

Prati, Daniele; Maggioni, Marco; Rebulla, Paolo; Fiorelli, G.; Coggi, Guido; Cappellini, Maria Domenica

doi:10.1016/s0168-8278(03)80830-9

Cited by 37 publications

(52 citation statements)

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“…Iron overload and HCV infection represent independent risk factors for progression of liver fibrosis in thalassemia patients following bone marrow transplantion [32], and data in transfusion-dependent thalassemia patients have shown a mild liver necroinflammation due to HCV infection but frequent significant fibrosis, the progression of which is largely influenced by iron overload [6].…”

Section: Discussionmentioning

confidence: 99%

Transient elastography in the assessment of liver fibrosis in adult thalassemia patients

et al. 2010

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Transient elastography (TE) is a valuable noninvasive technique of measuring liver stiffness and a reliable tool for predicting hepatic fibrosis in patients with chronic liver disease. The role of TE in patients with β‐thalassemia has not been extensively investigated. The present study aimed to evaluate the role of TE in the assessment of hepatic fibrosis in 115 adult patients with β‐thalassemia major (TM) (#59) or intermedia (TI) (#56). TE was performed according to current practice. Histologic data were obtained in 14 cases. Liver iron concentration was assessed by atomic absorption spectrometry and T2* magnetic resonance. In patients with TM, the proportion of anti‐HCV positive viremic patients, median serum ferritin levels, and TE values were significantly higher than in TI. In the group of 14 patients who underwent liver biopsy, a significant positive correlation was observed between liver stiffness and fibrosis stage (r = 0.73, P = 0.003). Severe fibrosis is diagnosed with a sensitivity of 60% and a specificity of 89%, whereas cirrhosis is detected with a sensitivity of 100% and a specificity of 92%. At multivariate analysis, the variables independently associated with TE were ALT, GGT, and bilirubin levels in both groups and, in patients with TM, HCV RNA positivity. In β‐thalassemia patients, TE is a reliable tool for assessing liver fibrosis even if the influence of iron overload has to be clarified. Am. J. Hematol. 85:564–568, 2010. © 2010 Wiley‐Liss, Inc.

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Section: Discussionmentioning

confidence: 99%

Transient elastography in the assessment of liver fibrosis in adult thalassemia patients

et al. 2010

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“…Until a few years ago, the liver biopsy was the only method available to assess the severity of liver inflammation, the stage of fibrosis, and to measure the liver iron concentration by atomic absorption spectrometry. [58][59][60][61] However, liver biopsy is an invasive procedure associated with some discomfort, and its accuracy for the evaluation of liver fibrosis is questionable in relation to inadequate tissue sampling and intraobserver and interobserver variability. 62 Finally, severe fibrosis or cirrhosis is responsible for significant variability in iron distribution in thalassemia patients.…”

Section: What Are the Currently Available Methods For Diagnosis Of VImentioning

confidence: 99%

“…[58][59][60][61]74,75 Male sex, high serum alanine transaminase values, positive serum HCV-RNA, and high liver iron concentration were all significantly associated with severe fibrosis or cirrhosis. [58][59][60][61] Cirrhosis is a risk factor for the development of HCC and is the major cause of liver failure. A multicenter cross-sectional Italian study reported data from 23 thalassemia patients receiving the diagnosis of HCC during the previous 20 years.…”

Section: Recommendations For Virologic and Clinical Evaluation Of Thamentioning

confidence: 98%

Management of chronic viral hepatitis in patients with thalassemia: recommendations from an international panel

Marco

Capra²,

Angelucci³

et al. 2010

Blood

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IntroductionIn the last 4 decades, regular blood transfusions and chelation therapy have improved the survival of patients with thalassemia major. 1-3 Despite the progress on chelation therapy, cardiac complications remain the main cause of death among transfusiondependent thalassemia patients related to the susceptibility of cardiac cells to iron overload toxicity. 4,5 The interest in the clinical management of chronic liver diseases has been increasing, however, because of the high prevalence of viral infections in adult transfusion-dependent thalassemia patients and the central role of the liver in regulating the iron metabolism. 5,6 The assessment of heart and liver iron overload is required to tailor iron chelation therapy. Furthermore, the diagnosis of hepatitis B virus (HBV)-or hepatitis C virus (HCV)-related chronic hepatitis is required to identify patients who have a high risk of developing liver complications and who may obtain a benefit by antiviral therapy.The goals of this paper are to summarize the epidemiology and the risks of transmission of viral infections, to analyze invasive and noninvasive methods for the diagnosis of chronic liver disease, to report the knowledge on clinical course of chronic viral hepatitis, and to suggest the management of antiviral therapy. For personal use only. on May 12, 2018. by guest www.bloodjournal.org From An expert hepatologist prepared an initial draft based on systematic review of published literature by Medline search on viral hepatitis in thalassemia patients, examined recently published guidelines on the diagnosis, management, and treatment of chronic hepatitis B and chronic hepatitis C edited by the American Association for the Study of Liver Diseases, the European Association for the Study of the Liver, and the Asian-Pacific Association for the Study of the Liver 7-10 and the Consensus Development Conference sponsored by the National Institutes of Health. 11 Recommendations of the panel of experts were based insofar as possible on evidence from publications that reported data on thalassemia patients. If evidence from thalassemia patients was unavailable, the panel selected recommendations from published guidelines that were suitable for thalassemia patients. Recommendations were evaluated according to the Grading of Recommendations Assessment Development and Evaluation system and classified into 3 levels: high, moderate, or low 12 (Table 1). For each recommendation, the level of evidence (high, moderate, or low) and population studied (general population of patients with viral chronic hepatitis or thalassemia patients) was specified. The panel of experts discussed the draft issue by issue, and 3 experts on blood transfusion, iron metabolism, and viral hepatitis management reviewed the recommendations. Finally, 8 international external experts in pediatrics, hematology, and viral hepatitis reviewed and criticized the recommendations. Methods Results What proportion of thalassemia patients have chronic viral infections?Worldwide, from 0.3% to 5.7% of thala...

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“…These cofactors are frequently encountered in post-transplant and have previously been shown to seriously compromise liver function and fibrogenesis. 15,16 They could be involved in a particular evolution of hepatitis C in post transplant 17 which is responsible for the large majority of subsequent cirrhosis. 18 Our selection criteria could underestimate the clinical impact of iron overload.…”

Section: Evolution After Phlebotomiesmentioning

confidence: 99%

Quantification by magnetic resonance imaging and liver consequences of post-transfusional iron overload alone in long-term survivors after allogeneic hematopoietic stem cell transplantation

Rosé¹,

Ernst²,

Hecquet³

et al. 2007

Haematologica

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Clinical and histological evaluation of liver disease in patients with transfusion-dependent beta-thalassemia: A multicenter study

Cited by 37 publications

References 0 publications

Transient elastography in the assessment of liver fibrosis in adult thalassemia patients

Transient elastography in the assessment of liver fibrosis in adult thalassemia patients

Management of chronic viral hepatitis in patients with thalassemia: recommendations from an international panel

Quantification by magnetic resonance imaging and liver consequences of post-transfusional iron overload alone in long-term survivors after allogeneic hematopoietic stem cell transplantation

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