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Use of the oral chelator deferiprone in the treatment of iron overload in patients with Hb H disease
Author(s) -
Chan Joyce C. W.,
Chim ChorSang,
Ooi Clara G. C.,
Cheung Bernard,
Liang Raymond,
Chan TaiKwong,
Chan Vivian
Publication year - 2006
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.2006.05984.x
Subject(s) - medicine , gastroenterology , deferiprone , diastole , ferritin , cardiac function curve , thalassemia , cardiology , surgery , blood pressure , heart failure
Summary Seventeen non‐transfusion‐dependent Chinese haemoglobin H (Hb H) disease patients (age 29–76 years) with serum ferritin >900 μ g/l were treated with deferiprone for up to 18 months. One patient withdrew and data from 16 patients were analysed. Sixteen other Hb H patients with ferritin <900 μ g/l, matched for age and genotype, acted as controls. Treatment was well tolerated except for mild arthralgia. Serum ferritin fell with treatment, reaching significance at 6 and 18 months (from 1492·3 ± 901·4 to 519·4 ± 405·4 μ g/l at 18 months, P = 0·0008). Nine of 16 patients had levels below 397 μ g/l before 18 months. Serum ferritin remained stable 6 months after stopping treatment. In contrast, there was no change in ferritin levels in the control group. Magnetic resonance imaging was used for measurement of liver iron content. Spin echo T 1 ‐signal intensity ratio (T 1 ‐SIR) and gradient echo T 2 ‐signal intensity ratio (T 2 ‐SIR) increased with treatment. T 2 ‐SIR rose from 0·17 ± 0·08 pretreatment to 0·58 ± 0·50 at 2 years ( P = 0·0055). Improvement occurred in 12 of 16 patients, reaching normal in three patients. Using echocardiography, peak early diastolic : late diastolic blood flow (E/A) remained unchanged with treatment, but isovolumic relaxation time (IVRT) was prolonged at 2 years indicating mild impairment of diastolic function. All systolic function parameters were normal. A longer treatment period is desirable to demonstrate improvement in cardiac function.