|Year : 2020 | Volume
| Issue : 2 | Page : 124-125
Extensive periportal fibrosis due to hepatic iron overload masquerading as mass lesions in a beta-thalassemia major patient: Sonological appearances
Reddy Ravikanth1, Denver Steven Pinto1, Pooja Majumdar2
1 Department of Radiology, St. John's Hospital, Kattappana, Kerala, India
2 Department of Medicine, INHS Kalyani, Visakhapatnam, Andhra Pradesh, India
|Date of Submission||07-May-2020|
|Date of Acceptance||16-May-2020|
|Date of Web Publication||18-Jun-2020|
Department of Radiology, St. John's Hospital, Kattappana - 685 515, Kerala
Source of Support: None, Conflict of Interest: None
Beta-thalassemia major as a condition is dependent on multiple blood transfusions. In patients with multiple blood transfusions, the liver is the initial site of iron deposition progressing from fibrosis to cirrhosis. Ultrasonography diagnosis of liver fibrosis has prognostic significance and is helpful in risk stratification for assessing treatment options. This case report describes the sonological appearances of the liver in a 28-year-old female who was a recipient of regular blood transfusions and subsequently developed hepatic iron overload which presented as extensive periportal fibrosis masquerading as intraparenchymal mass lesions.
Keywords: Beta-thalassemia major, hepatic hemosiderosis, liver biopsy, multiple blood transfusions, periportal fibrosis, ultrasonography
|How to cite this article:|
Ravikanth R, Pinto DS, Majumdar P. Extensive periportal fibrosis due to hepatic iron overload masquerading as mass lesions in a beta-thalassemia major patient: Sonological appearances. Apollo Med 2020;17:124-5
|How to cite this URL:|
Ravikanth R, Pinto DS, Majumdar P. Extensive periportal fibrosis due to hepatic iron overload masquerading as mass lesions in a beta-thalassemia major patient: Sonological appearances. Apollo Med [serial online] 2020 [cited 2020 Jul 8];17:124-5. Available from: http://www.apollomedicine.org/text.asp?2020/17/2/124/287092
| Introduction|| |
In transfusion-dependent β-thalassemia major, the main causes of liver fibrosis are hepatic iron overload and hepatitis C virus (HCV) infection. Young β-thalassemia major patients are likely to develop hepatic fibrosis and end-stage liver cirrhosis accompanied by hepatic hemosiderosis. This case report describes the sonological appearances of the liver in a 28-year-old female who was a recipient of regular blood transfusions and subsequently developed hepatic iron overload which presented as extensive periportal fibrosis masquerading as intraparenchymal mass lesions.
| Case Report|| |
A 28-year-old female presented to the casualty with complaints of acute onset pain in the right hypochondrium for 24 h. She is a known case of beta-thalassemia major and underwent splenectomy 6 months ago. She was a recipient of regular blood transfusions to achieve hemoglobin goal of 10 g%, and the last transfusion was but a week ago. She was referred for abdominal ultrasonography which revealed hepatomegaly with coarsened parenchymal echotexture and echogenic thickening of portovenous bundles along the main portal vein and its branches [Figure 1]a. On initial screening, the periportal echogenicities resembled echogenic mass lesions scattered in the parenchyma of both lobes of the liver [Figure 1]b. However, they were actually linear echogenicities along the portal tracts which had vascularity on color Doppler examination which was evident of periportal fibrosis in the background of chronic liver disease [Figure 1]c. Transient elastography is performed with FibroScan (Echosens, Paris, France) which is the most validated method of ultrasound elastography for the noninvasive diagnosis of liver fibrosis. FibroScan performed on the patient had a stiffness value of 10 kPa (cutoff value >7 kPa for significant fibrosis). Magnetic resonance imaging demonstrated periportal signal drop on in-phase image as compared to out-of-phase image in the periportal area due to periportal iron accumulation with resultant fibrosis. She had elevated levels of ferritin which is due to regular blood transfusions, and a diagnosis of extensive liver fibrosis secondary to hepatic iron overload was made.
|Figure 1: Longitudinal ultrasonography image demonstrating multiple echogenic mass-like lesions (stars) scattered in the segments of both lobes of the liver. (a) Note the serpigineous echogenic thickening along the portovenous bundles (arrows); (b) and color uptake on Doppler examination evident of periportal fibrosis (c)|
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| Discussion|| |
Beta-thalassemia major as a condition is dependent on multiple blood transfusions. In patients with multiple blood transfusions, liver is the initial site of iron deposition progressing from fibrosis to cirrhosis. Liver fibrosis is directly dependent on liver iron concentration and the number of blood units transfused. Excess iron can induce fibrosis in periportal regions and parenchyma, which accelerate disease progression and can exacerbate liver pathology.
Ultrasonography diagnosis of liver fibrosis has prognostic significance and is helpful in risk stratification for assessing treatment options. Quantitative elastography is an objective and noninvasive alternative diagnosis for liver biopsy. Liver biopsy, due to its invasiveness and being a costly investigation is impractical as a reference standard for monitoring treatment response in patients with liver fibrosis. However, detection or quantification of liver iron overload on ultrasonography is a major limitation.
Hereditary hemochromatosis caused by (HFE) gene mutations is a cause of primary iron overload. The conditions such as β-thalassemia major, sickle cell disease, myelodysplasic syndromes, aplastic anemia, and Blackfan-Diamond anemia which are associated with multiple red blood cell transfusions are the causes for secondary iron overload. Currently, hematopoietic stem cell transplantation is the only curative option for patients with beta-thalassemia major.
Both primary and secondary iron overload can lead to diffuse hepatic iron overload. However, diffuse siderosis is mostly observed in patients receiving multiple blood transfusions. Excess iron due to increased intestinal absorption initially accumulates in the periportal hepatocytes. Therefore, periportal siderosis can be observed in the early phases of hepatic iron overload.
Hyperechoic periportal cuffings are seen as thick echogenic bands around the portovenous bundles in the periportal connective tissue of the portal triad and are noted in hepatic hemosiderosis in β-thalassemia major, frequently associated with hepatomegaly, coarsening of echotexture and a diffusely decreased echogenicity of the parenchyma that determines a relative increase in the echogenicity of the portal vein walls and explains the sonographic appearance of the liver in “starry sky” or centrilobular pattern. Ultrasound-based elastography, tissue elastography, and acoustic radiation force impulse have been developed to assess liver fibrosis.
| Conclusion|| |
Liver fibrosis is an important entity as its progression can lead to organ failure and hepatocellular carcinoma where currently resection or transplantation are the only curative options. The final stage of liver fibrosis is cirrhosis and often leads to portal hypertension and hepatic failure. Hence, early identification on ultrasonography carries a prognostic significance and is a reliable investigation for assessing treatment options.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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