Liver complications of haemoglobin H disease in adults
Corresponding Author
Luke K. L. Chan
Division of Haematology, Department of Medicine and Geriatrics, Princess Margaret Hospital, Hong Kong, China
Correspondence: Luke K. L. Chan, Resident Specialist, Division of Haematology, Department of Medicine and Geriatrics, Princess Margaret Hospital, Princess Margaret Hospital Road, Kwai Chung, Hong Kong, China.
E-mail: [email protected]
Search for more papers by this authorVivien W. M. Mak
Division of Haematology, Department of Medicine and Geriatrics, Princess Margaret Hospital, Hong Kong, China
Search for more papers by this authorStanley C. H. Chan
Department of Radiology, Princess Margaret Hospital, Hong Kong, China
Search for more papers by this authorEllen L. M. Yu
Clinical Research Centre, Princess Margaret Hospital, Hong Kong, China
Search for more papers by this authorNelson C. N. Chan
Department of Anatomical and Cellular Pathology, Prince of Wales Hospital, The Chinese University of Hong Kong, Hong Kong, China
Search for more papers by this authorKate F. S. Leung
Division of Haematology, Department of Pathology, Princess Margaret Hospital, Hong Kong, China
Search for more papers by this authorCarmen K. M. Ng
Division of Gastroenterology and Hepatology, Department of Medicine and Geriatrics, Princess Margaret Hospital, Hong Kong, China
Search for more papers by this authorMargaret H. L. Ng
Department of Anatomical and Cellular Pathology, Prince of Wales Hospital, The Chinese University of Hong Kong, Hong Kong, China
Search for more papers by this authorJoyce C. W. Chan
Division of Haematology, Department of Medicine and Geriatrics, Princess Margaret Hospital, Hong Kong, China
Search for more papers by this authorHarold K. K. Lee
Division of Haematology, Department of Medicine and Geriatrics, Princess Margaret Hospital, Hong Kong, China
Search for more papers by this authorCorresponding Author
Luke K. L. Chan
Division of Haematology, Department of Medicine and Geriatrics, Princess Margaret Hospital, Hong Kong, China
Correspondence: Luke K. L. Chan, Resident Specialist, Division of Haematology, Department of Medicine and Geriatrics, Princess Margaret Hospital, Princess Margaret Hospital Road, Kwai Chung, Hong Kong, China.
E-mail: [email protected]
Search for more papers by this authorVivien W. M. Mak
Division of Haematology, Department of Medicine and Geriatrics, Princess Margaret Hospital, Hong Kong, China
Search for more papers by this authorStanley C. H. Chan
Department of Radiology, Princess Margaret Hospital, Hong Kong, China
Search for more papers by this authorEllen L. M. Yu
Clinical Research Centre, Princess Margaret Hospital, Hong Kong, China
Search for more papers by this authorNelson C. N. Chan
Department of Anatomical and Cellular Pathology, Prince of Wales Hospital, The Chinese University of Hong Kong, Hong Kong, China
Search for more papers by this authorKate F. S. Leung
Division of Haematology, Department of Pathology, Princess Margaret Hospital, Hong Kong, China
Search for more papers by this authorCarmen K. M. Ng
Division of Gastroenterology and Hepatology, Department of Medicine and Geriatrics, Princess Margaret Hospital, Hong Kong, China
Search for more papers by this authorMargaret H. L. Ng
Department of Anatomical and Cellular Pathology, Prince of Wales Hospital, The Chinese University of Hong Kong, Hong Kong, China
Search for more papers by this authorJoyce C. W. Chan
Division of Haematology, Department of Medicine and Geriatrics, Princess Margaret Hospital, Hong Kong, China
Search for more papers by this authorHarold K. K. Lee
Division of Haematology, Department of Medicine and Geriatrics, Princess Margaret Hospital, Hong Kong, China
Search for more papers by this authorAbstract
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.
Conflict of interests
The authors declare there are no conflicts of interests specific to this submission.
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