Чернодробни усложнения при ß-таласемия

  • Деница Кофинова
Keywords: таласемия, черен дроб, усложнения

Abstract

Бета-таласемията е едно от най-честите автозомно-рецесивни заболявания. С подобряване на хелаторното лечение и навлизането на неинвазивните методики за оценка и проследяване на желязното натрупването, качеството и продължителността на живот на пациентите с ß-таласемия значително се подобри. Черният дроб е първия таргетен орган, който се уврежда, директно и индиректно. В настоящия обзор се разглеждат най-честите чернодробни усложнения като развитие на фиброза и цироза, методи за оценка на желязното натоварване, хронични вирусни хепатити, развитие на хепатоцелуларен карцином и холелитиаза.

References

Modell B, Darlison M. Global epidemiology of haemoglobin disorders and derived service indicators. Bull World Health Organ. 2008;86:480-487
Kattamis A, Forni GL, Aydinok Y, et al. Changing patterns in the epidemiology of β-thalassemia. Eur J Haematol. 2020 Dec;105(6):692-703.
Георгиев П. Дисертационен труд. Ефект на хелатиращата терапия върху желязното свръхнатоварване, чернодробната и сърдечна дисфункции при възрастни пациенти с трансфузионно зависима β –таласемия. МУ-Пловдив 2020, 23
Cappellini MD, Cohen A, Eleftheriou A et al. Guidelines for the Clinical Management of Thalassaemia [Internet]. 2nd Revised ed. Nicosia (CY): Thalassaemia International Federation; 2008
Angelucci E, Baronciani D, Lucarelli G, et al. Needle liver biopsy in thalassaemia: analyses of diagnostic accuracy and safety in 1184 consecutive biopsies. British Journal of Haematology 1995; 89: 757–61.
Dezsőfi A, Baumann U, Dhawan A, et al. Liver biopsy in children: position paper of the ESPGHAN Hepatology Committee. J Pediatr Gastroenterol Nutr. 2015 Mar;60(3):408-20.
Хаджийски П. За и против чернодробната биопсия в детска възраст. Педиатрия 2020;60 (3):15-18.
Georgiev PG, Sapunarova KG, Goranova-Marinova VS, et al. Reduction of Liver Iron Load in Adult Patients with β-Thalassemia Major Treated with Modern Chelation Modalities. Folia Med (Plovdiv). 2020 Jun 30;62(2):265-270.
Bonkovsky HL, Rubin RB, Cable EE, et al. Hepatic iron concentra¬tion: noninvasive estimation by means of MR imaging technique. Radiology 1999; 212: 227–34.
Kanbour I, Chandra P, Soliman A, et al. Severe liver iron concen¬trations (LIC) in 24 patients with β-thalassemia major: correlations with serum ferritin, liver enzymes and endocrine complications. Mediterr J Hematol Infect Dis 2018; 10(1): e2018062.
Porter JB, Elalfy M, Taher A, et al. Limitations of serum ferritin to predict liver iron concentration responses to deferasirox therapy in patients with transfusion-dependent thalassaemia. Eur J Haematol 2017; 98(3): 280–8.
Jarisch A, Salzmann-Manrique E, Cario H, et al. Serum ferritin is not a reliable predictor to determine iron overload in thalassemia major patients post-hematopoietic stem cell transplantation. Eur J Haematol 2018; 101(6): 791–7.
Taher A, Nathan D, Porter JD. Evaluation of iron levels to avoid the clinical sequelae of iron overload. Semin Hematol 2007; 44 (Suppl 3): S2–S6.
The Cooley’s Anemia Foundation. Position statement on MRI based hepatic iron assessment methods. http://www.thalassemia.org/2012-position-statement-on-mri-based-hepatic-iron-assessment-methods-2
Yang G, Liu R, Peng P, et al. How early can myocardialiron overload occur in beta thalassemia major? PLoSOne.2014;9:e85379.
Berdoukas V, Nord A, Carson S, et al. Tissue iron evalu-ation in chronically transfused children shows significant levels of iron loading at a very young age. Am JHematol.2013;88:E283–E285.
Хаджиниколова М, Енчев Д, Балтов А и съавт. Ултразвукова еластография – видове, физични аспекти на методиката и клинично приложение при опорно-двигателна система. Диагностичен и терапевтичен ултразвук.2022;60(1):10-18.
Комитова Т, Митова Р, Генов Й и съавт. Национални правила и препоръки за провеждане на ултразвукова еластография на черен дроб. Диагностичен и терапевтичен ултразвук.2020;28(1):3-11.
Di Marco V, Bronte F, Cabibi D et al. Noninvasive assessment of liver fibrosis in thalassaemia major patients by transient elastography (TE) - lack of interference by iron deposition. Br J Haematol. 2010 Feb;148(3):476-9.
Pipaliya N, Solanke D, Parikh P, et al. Comparison of Tissue Elastography With Magnetic Resonance Imaging T2* and Serum Ferritin Quantification in Detecting Liver Iron Overload in Patients With Thalassemia Major. Clin Gastroenterol Hepatol. 2017 Feb;15(2):292-298.e1
Paparo F, Cevasco L, Zefiro D et al. Diagnostic value of real-time elastography in the assessment of hepatic fibrosis in patients with liver iron overload. Eur J Radiol. 2013 Dec;82(12):e755-61.
Al-Khabori M, Daar S, Al-Busafi SA, et al. Noninvasive assessment and risk factors of liver fibrosis in patients with thalassemia major using shear wave elastography. Hematology. 2019 Dec;24(1):183-188.
Prati D, Maggioni M, Milani S, et al. Clinical and histological characterization of liver disease in patients with transfusion-dependent beta-thalassemia. A multicenter study of 117 cases. Haematologica 2004;89:1179–1186.
Rigamonti C, Andorno S, Maduli E, et al. Gender and liver fibrosis in chronic hepatitis: the role of iron status. Aliment Pharmacol Ther. 2005;21:1445–1451.
Marsella M, Borgna-Pignatti C, Meloni A, et al. Cardiac iron and cardiac disease in males and females with transfusion-dependent thalassemia major: a t2* magnetic resonance imaging study. Haematologica. 2011;96:515–520.
Taher AT, Cappellini MD. How I manage medical complications of β-thalassemia in adults. Blood. 2018 Oct 25;132(17):1781-1791.
Хаджийски П, Байчева М, Найденов Х и съавт. Иновативно лечение на хроничния С хепатит при децата с пангенотипната комбинация Glecaprevir/Pibrentasvir – невъзможното вчера- възможно днес. Педиатрия 2022;62 (2): 37-39.
Rumi MG, Di Marco V, Colombo M. Management of HCV-related liver disease in hemophilia and thalassemia. Semin Liver Dis. 2018;38(2):112-120.
Di Marco V, Capra M, Angelucci E, et al. Italian Society for the Study of Thalassemia and Haemoglobinopathies; Italian Association for the Study of the Liver. Management of chronic viral hepatitis in patients with thalassemia: recommendations from an international panel. Blood. 2010 Oct 21;116(16):2875-83.
Angelucci E, Muretto P, Nicolucci A, et al. Effects of iron overload and hepatitis C virus positivity in determining progression of liver fibrosis in thalassemia following bone marrow transplantation. Blood. 2002;100(1):17-21.
European Association for the Study of the Liver. EASL recommendations on treatment of hepatitis C: Final update of the series. J Hepatol. 2020 Nov;73(5):1170-1218.
Di Serafino M, Severino R, Gioioso M, et al. Paediatric liver ultrasound: a pictorial essay. J Ultrasound. 2020 Mar;23(1):87-103
Moukhadder HM, Halawi R, Cappellini MD, et al. Hepatocellular carcinoma as an emerging morbidity in the thalassemia syndromes: A comprehensive review. Cancer. 2017 Mar 1;123(5):751-758
De Sanctis V, Soliman AT, Daar S, et al. A Concise Review on the Frequency, Major Risk Factors and Surveillance of Hepatocellular Carcinoma (HCC) in β-Thalassemias: Past, Present and Future Perspectives and the ICET-A Experience. Mediterr J Hematol Infect Dis. 2020 Jan 1;12(1):e2020006.
Origa R, Galanello R, Perseu L, et al. Cholelithiasis in thalassemia major. Eur J Haematol. 2009 Jan;82(1):22-5.
Published
2023-09-13
How to Cite
1.
Кофинова Д. Чернодробни усложнения при ß-таласемия. Редки болести и лекарства сираци [Internet]. 2023Sep.13 [cited 2024Jul.22];14(1):5-. Available from: https://journal.raredis.org/index.php/RBLS/article/view/172
Section
Статии