Liver complications of haemoglobin H disease in adults.

Autor: Chan LKL; Division of Haematology, Department of Medicine and Geriatrics, Princess Margaret Hospital, Hong Kong, China., Mak VWM; Division of Haematology, Department of Medicine and Geriatrics, Princess Margaret Hospital, Hong Kong, China., Chan SCH; Department of Radiology, Princess Margaret Hospital, Hong Kong, China., Yu ELM; Clinical Research Centre, Princess Margaret Hospital, Hong Kong, China., Chan NCN; Department of Anatomical and Cellular Pathology, Prince of Wales Hospital, The Chinese University of Hong Kong, Hong Kong, China., Leung KFS; Division of Haematology, Department of Pathology, Princess Margaret Hospital, Hong Kong, China., Ng CKM; Division of Gastroenterology and Hepatology, Department of Medicine and Geriatrics, Princess Margaret Hospital, Hong Kong, China., Ng MHL; Department of Anatomical and Cellular Pathology, Prince of Wales Hospital, The Chinese University of Hong Kong, Hong Kong, China., Chan JCW; Division of Haematology, Department of Medicine and Geriatrics, Princess Margaret Hospital, Hong Kong, China., Lee HKK; Division of Haematology, Department of Medicine and Geriatrics, Princess Margaret Hospital, Hong Kong, China.
Jazyk: angličtina
Zdroj: British journal of haematology [Br J Haematol] 2021 Jan; Vol. 192 (1), pp. 171-178. Date of Electronic Publication: 2020 Oct 23.
DOI: 10.1111/bjh.17115
Abstrakt: Haemoglobin H (HbH) disease is a type of non-transfusion-dependent thalassaemia. This cross-sectional study aimed at determining the prevalence and severity of liver iron overload and liver fibrosis in patients with HbH disease. Risk factors for advanced liver fibrosis were also identified. A total of 80 patients were evaluated [median (range) age 53 (24-79) years, male 34%, non-deletional HbH disease 24%]. Patients underwent 'observed' T2-weighted magnetic resonance imaging examination for liver iron concentration (LIC) quantification, and transient elastography for liver stiffness measurement (LSM) and fibrosis staging. In all, 25 patients (31%) had moderate-to-severe liver iron overload (LIC ≥7 mg/g dry weight). The median LIC was higher in non-deletional than in deletional HbH disease (7·8 vs. 2.9 mg/g dry weight, P = 0·002). In all, 16 patients (20%) had advanced liver fibrosis (LSM >7.9 kPa) and seven (9%) out of them had probable cirrhosis (LSM >11.9 kPa). LSM positively correlated with age (R = 0·24, P = 0·03), serum ferritin (R = 0·36, P = 0·001) and LIC (R = 0·28, P = 0·01). In multivariable regression, age ≥65 years [odds ratio (OR) 4·97, 95% confidence interval (CI) 1·52-17·50; P = 0·047] and moderate-to-severe liver iron overload (OR 3·47, 95% CI 1·01-12·14; P = 0·01) were independently associated with advanced liver fibrosis. The findings suggest that regular screening for liver complications should be considered in the management of HbH disease.
(© 2020 British Society for Haematology and John Wiley & Sons Ltd.)
Databáze: MEDLINE
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