Isolated focal intrahepatic extramedullary hematopoiesis mimicking hepatocellular carcinoma in cirrhotic patient with secondary hemochromatosis from thalassemia
Gastroenterology and Hepatology from Bed to Bench,
Vol. 16 No. 1 (2023),
2 January 2023
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.
- Extramedullary hematopoiesis
- Thalassemia
- Hemochromatosis
- Hepatocellular carcinoma
How to Cite
References
Kim CH. Homeostatic and pathogenic extramedullary hematopoiesis. J Blood Med 2010;1:13–19.
Bao Y, Liu Z, Guo M, Li B, Sun X, Wang L. Extramedullary hematopoiesis secondary to malignant solid tumors: a case report and literature review. Cancer Manag Res 2018;10:1461–1470.
Yang X, Chen D, Long H, Zhu B. The mechanisms of pathological extramedullary hematopoiesis in diseases. Cell Mol Life Sci 2020;77:2723–2738.
Yamamoto K, Miwa Y, Abe-Suzuki S, Abe S, Kirimura S, Onishi I, et al. Extramedullary hematopoiesis: Elucidating the function of the hematopoietic stem cell niche (Review). Mol Med Rep 2016;13:587–591.
Thonglairuam V, Winichagoon P, Fucharoen S, Wasi P. The molecular basis of AE-Bart’s disease. Hemoglobin 1989;13:117–124.
Musallam KM, Rivella S, Vichinsky E, Rachmilewitz EA. Non-transfusion-dependent thalassemias. Haematologica 2013;98:833–844.
Taher A, Isma'eel H, Cappellini MD. Thalassemia intermedia: revisited. Blood Cells Mol Dis 2006;37:12–20.
Tamiolakis D, Venizelos J, Prassopoulos P, Simopoulos S, Bolioti S, Tsiapali M, et al. Intrahepatic extramedullary hematopoietic tumor mimicking metastatic carcinoma from a colonic primary. Onkologie 2004;27:65–67.
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.
Kopecky KK, Moriarty AT, Antony AC, Baker MK. Extramedullary hematopoiesis in acute lymphocytic leukemia masquerading as hepatic, renal, and splenic microabscesses. AJR Am J Roentgenol 1986;147:846–847.
Navarro M, Crespo C, Pérez L, Martínez C, Galant J, González I. Massive intrahepatic extramedullary hematopoiesis in myelofibrosis. Abdom Imaging 2000;25:184–186.
Bradley MJ, Metreweli C. Ultrasound appearances of extramedullary haematopoiesis in the liver and spleen. Br J Radiol 1990;63:816–818.
Wong Y, Chen F, Tai KS, Yip LK, Tsang KW, Chan FL, et al. Imaging features of focal intrahepatic extramedullary haematopoiesis. Br J Radiol 1999;72:906–910.
Priola AM, Gned D, Boccuzzi F, Priola SM. Unusual focal intrahepatic extramedullary haematopoiesis in alpha-thalassaemia. Liver Int 2012;32:771.
Shakeri R, Rahmati A, Zamani F. Solitary huge intrahepatic mass (extramedullary hematopoiesis). Arch Iran Med 2013;16:315–316.
Mancuso A. Hepatocellular carcinoma in thalassemia: a critical review. World J Hepatol 2010;2:171–174.
Mancuso A, Sciarrino E, Renda MC, Maggio A. A prospective study of hepatocellular carcinoma incidence in thalassemia. Hemoglobin 2006;30:119–124.
Musallam KM, Cappellini MD, Wood JC, Motta I, Graziadei G, Tamim H, et al. Elevated liver iron concentration is a marker of increased morbidity in patients with β thalassemia intermedia. Haematologica 2011;96:1605–1612.
Jean G, Terzoli S, Mauri R, Borghetti L, Di Palma A, Piga A, et al. Cirrhosis associated with multiple transfusions in thalassaemia. Arch Dis Child 1984;59:67-70.
Fragatou S, Tsourveloudis I, Manesis G. Incidence of hepatocellular carcinoma in a thalassemia unit. Hemoglobin 2010;34:221–226.
Lefkowitch JH. Hepatobiliary pathology. Curr Opin Gastroenterol 2006;22:198–208.
Tsochatzis EA, Bosch J, Burroughs AK. Liver cirrhosis Lancet. 2014;383:1749–1761.
De Tomás J, Louredo A, Turégano F, Alonso A, Martín-Merino MR. Hepatocellular carcinoma in a non-cirrhotic coal miner with secondary haemochromatosis. Eur J Gastroenterol Hepatol 1997;9:633–634.
Chung H, Kudo M, Kawasaki T, Kitano M, Minami Y, Suetomi Y ,et al. Hepatocellular carcinoma associated with secondary haemochromatosis in non-cirrhotic liver: a case report. Hepatol Res 2003;26:254–258.
Ikoma N, Shinozaki H, Kozuki A, Ibuki S, Sugano K,Mukai M, 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;4:248–251.
Yamauchi R, Takata K, Shinagawa Y, Tanaka T, Fukuda H, Fukuda S,et al. Hepatocellular carcinoma arising in a non-cirrhotic liver with secondary hemochromatosis. Intern Med 2019;58:661–665.
Clark T, Maximin S, Meier J, Pokharel S, Bhargava P. Hepatocellular carcinoma: review of epidemiology, screening, imaging diagnosis, response assessment, and treatment. Curr Probl Diagn Radiol 2015;44:479–486.
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