ISSN: 2008-2258
Vol 16, No 1 (2023): Winter
Isolated focal intrahepatic extramedullary hematopoiesis mimicking hepatocellular carcinoma in cirrhotic patient with secondary hemochromatosis from thalassemia
Gastroenterology and Hepatology from Bed to Bench,
,
https://doi.org/10.22037/ghfbb.v16i1.2696
Abstract
Extramedullary hematopoiesis is a common complication of ineffective erythropoiesis and bone marrow replacement disorders. Owing to its nonspecific presentation and or radiological appearances, diagnosing focal intrahepatic extramedullary hematopoiesis is challenging, and it is often misdiagnosed as a hepatic tumor. Herein, we describe the case of a 48-year-old male with thalassemia and AE Bart’s disease with secondary hemochromatosis and cirrhosis who developed focal intrahepatic extramedullary hematopoiesis mimicking hepatocellular carcinoma. After hepatic resection, extramedullary hematopoiesis was not observed at any site, including in the remaining liver, at 4-year follow-up.
Keywords:
- Extramedullary hematopoiesis
- Thalassemia
- Hemochromatosis
- Hepatocellular carcinoma
How to Cite
Sattayaraksa, T., Charoenlap, C., Akarapatima, K., Rattanasupar, A., & Chang, A. (2022). Isolated focal intrahepatic extramedullary hematopoiesis mimicking hepatocellular carcinoma in cirrhotic patient with secondary hemochromatosis from thalassemia. Gastroenterology and Hepatology from Bed to Bench, 16(1). https://doi.org/10.22037/ghfbb.v16i1.2696
References
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2. Bao Y, Liu Z, Guo M, et al. Extramedullary hematopoiesis secondary to malignant solid tumors: a case report and literature review. Cancer Manag Res. 2018;10:1461–70.
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8. Tamiolakis D, Venizelos J, Prassopoulos P, et al. Intrahepatic extramedullary hematopoietic tumor mimicking metastatic carcinoma from a colonic primary. Onkologie. 2004;27:65–7.
9. Kumar A, Aggarwal S, de Tilly LN. Case of the season. Thalassemia major with extramedullary hematopoiesis in the liver. Semin Roentgenol. 1995;30:99–101.
10. Kopecky KK, Moriarty AT, Antony AC, et al. Extramedullary hematopoiesis in acute lymphocytic leukemia masquerading as hepatic, renal, and splenic microabscesses. AJR Am J Roentgenol. 1986;147:846–7.
11. Navarro M, Crespo C, Pérez L, et al. Massive intrahepatic extramedullary hematopoiesis in myelofibrosis. Abdom Imaging. 2000;25:184–6.
12. Bradley MJ, Metreweli C. Ultrasound appearances of extramedullary haematopoiesis in the liver and spleen. Br J Radiol. 1990;63:816–8.
13. Wong Y, Chen F, Tai KS, et al. Imaging features of focal intrahepatic extramedullary haematopoiesis. Br J Radiol. 1999;72:906–10.
14. Priola AM, Gned D, Boccuzzi F, et al. Unusual focal intrahepatic extramedullary haematopoiesis in alpha-thalassaemia. Liver Int. 2012;32:771.
15. Shakeri R, Rahmati A, Zamani F. Solitary huge intrahepatic mass (extramedullary hematopoiesis). Arch Iran Med. 2013;16:315–6.
16. Mancuso A. Hepatocellular carcinoma in thalassemia: A critical review. World J Hepatol. 2010;2:171–4.
17. Mancuso A, Sciarrino E, Renda MC, et al. A prospective study of hepatocellular carcinoma incidence in thalassemia. Hemoglobin. 2006;30:119–24.
18. Musallam KM, Cappellini MD, Wood JC, et al. Elevated liver iron concentration is a marker of increased morbidity in patients with β thalassemia intermedia. Haematologica. 2011;96:1605–12.
19. Jean G, Terzoli S, Mauri R, et al. Cirrhosis associated with multiple transfusions in thalassaemia. Arch Dis Child. 1984;59:67–70.
20. Fragatou S, Tsourveloudis I, Manesis G. Incidence of hepatocellular carcinoma in a thalassemia unit. Hemoglobin. 2010;34:221–6.
21. Lefkowitch JH. Hepatobiliary pathology. Curr Opin Gastroenterol. 2006;22:198–208.
22. Tsochatzis EA, Bosch J, Burroughs AK. Liver cirrhosis. Lancet. 2014;383:1749–61.
23. De Tomás J, Louredo A, Turégano F, et al. Hepatocellular carcinoma in a non-cirrhotic coal miner with secondary haemochromatosis. Eur J Gastroenterol Hepatol. 1997;9:633–4.
24. Chung H, Kudo M, Kawasaki T, et al. Hepatocellular carcinoma associated with secondary haemochromatosis in non-cirrhotic liver: a case report. Hepatol Res. 2003;26:254–8.
25. Ikoma N, Shinozaki H, Kozuki A, et al. A case report of hepatocellular carcinoma in a non-cirrhotic patient with liver iron overload associated with myelodysplastic syndrome. World J Oncol. 2013;4:248–51.
26. Yamauchi R, Takata K, Shinagawa Y, et al. Hepatocellular carcinoma arising in a non-cirrhotic liver with secondary hemochromatosis. Intern Med. 2019;58:661–5.
27. Clark T, Maximin S, Meier J, et al. Hepatocellular carcinoma: review of epidemiology, screening, imaging diagnosis, response assessment, and treatment. Curr Probl Diagn Radiol. 2015;44:479–86.
2. Bao Y, Liu Z, Guo M, et al. Extramedullary hematopoiesis secondary to malignant solid tumors: a case report and literature review. Cancer Manag Res. 2018;10:1461–70.
3. Yang X, Chen D, Long H, et al. The mechanisms of pathological extramedullary hematopoiesis in diseases. Cell Mol Life Sci. 2020;77:2723–38.
4. Yamamoto K, Miwa Y, Abe-Suzuki S, et al. Extramedullary hematopoiesis: Elucidating the function of the hematopoietic stem cell niche (Review). Mol Med Rep. 2016;13:587–91.
5. Thonglairuam V, Winichagoon P, Fucharoen S, et al. The molecular basis of AE-Bart's disease. Hemoglobin. 1989;13:117–24.
6. Musallam KM, Rivella S, Vichinsky E, et al. Non-transfusion-dependent thalassemias. Haematologica. 2013;98:833–44.
7. Taher A, Isma'eel H, Cappellini MD. Thalassemia intermedia: revisited. Blood Cells Mol Dis. 2006;37:12–20.
8. Tamiolakis D, Venizelos J, Prassopoulos P, et al. Intrahepatic extramedullary hematopoietic tumor mimicking metastatic carcinoma from a colonic primary. Onkologie. 2004;27:65–7.
9. Kumar A, Aggarwal S, de Tilly LN. Case of the season. Thalassemia major with extramedullary hematopoiesis in the liver. Semin Roentgenol. 1995;30:99–101.
10. Kopecky KK, Moriarty AT, Antony AC, et al. Extramedullary hematopoiesis in acute lymphocytic leukemia masquerading as hepatic, renal, and splenic microabscesses. AJR Am J Roentgenol. 1986;147:846–7.
11. Navarro M, Crespo C, Pérez L, et al. Massive intrahepatic extramedullary hematopoiesis in myelofibrosis. Abdom Imaging. 2000;25:184–6.
12. Bradley MJ, Metreweli C. Ultrasound appearances of extramedullary haematopoiesis in the liver and spleen. Br J Radiol. 1990;63:816–8.
13. Wong Y, Chen F, Tai KS, et al. Imaging features of focal intrahepatic extramedullary haematopoiesis. Br J Radiol. 1999;72:906–10.
14. Priola AM, Gned D, Boccuzzi F, et al. Unusual focal intrahepatic extramedullary haematopoiesis in alpha-thalassaemia. Liver Int. 2012;32:771.
15. Shakeri R, Rahmati A, Zamani F. Solitary huge intrahepatic mass (extramedullary hematopoiesis). Arch Iran Med. 2013;16:315–6.
16. Mancuso A. Hepatocellular carcinoma in thalassemia: A critical review. World J Hepatol. 2010;2:171–4.
17. Mancuso A, Sciarrino E, Renda MC, et al. A prospective study of hepatocellular carcinoma incidence in thalassemia. Hemoglobin. 2006;30:119–24.
18. Musallam KM, Cappellini MD, Wood JC, et al. Elevated liver iron concentration is a marker of increased morbidity in patients with β thalassemia intermedia. Haematologica. 2011;96:1605–12.
19. Jean G, Terzoli S, Mauri R, et al. Cirrhosis associated with multiple transfusions in thalassaemia. Arch Dis Child. 1984;59:67–70.
20. Fragatou S, Tsourveloudis I, Manesis G. Incidence of hepatocellular carcinoma in a thalassemia unit. Hemoglobin. 2010;34:221–6.
21. Lefkowitch JH. Hepatobiliary pathology. Curr Opin Gastroenterol. 2006;22:198–208.
22. Tsochatzis EA, Bosch J, Burroughs AK. Liver cirrhosis. Lancet. 2014;383:1749–61.
23. De Tomás J, Louredo A, Turégano F, et al. Hepatocellular carcinoma in a non-cirrhotic coal miner with secondary haemochromatosis. Eur J Gastroenterol Hepatol. 1997;9:633–4.
24. Chung H, Kudo M, Kawasaki T, et al. Hepatocellular carcinoma associated with secondary haemochromatosis in non-cirrhotic liver: a case report. Hepatol Res. 2003;26:254–8.
25. Ikoma N, Shinozaki H, Kozuki A, et al. A case report of hepatocellular carcinoma in a non-cirrhotic patient with liver iron overload associated with myelodysplastic syndrome. World J Oncol. 2013;4:248–51.
26. Yamauchi R, Takata K, Shinagawa Y, et al. Hepatocellular carcinoma arising in a non-cirrhotic liver with secondary hemochromatosis. Intern Med. 2019;58:661–5.
27. Clark T, Maximin S, Meier J, et al. Hepatocellular carcinoma: review of epidemiology, screening, imaging diagnosis, response assessment, and treatment. Curr Probl Diagn Radiol. 2015;44:479–86.
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