Arthropathy in thalassaemia patients receiving deferiprone

Posterior subcapsular opacity in two patients with thalassaemia major following deferiprone consumption

“…5,6 This deferiprone-induced shift of iron may also be responsible for iron deposition and subcapsular opacities in the lens, as is presumed in the cases reported here. In addition, as a small water-soluble compound with a molecular weight of only 139 daltons (compared to deferoxamine with a molecular weight of 561 daltons), deferiprone would be expected to move freely through cell membranes all over the body.…”

“…More common but less serious side-effects are gastrointestinal symptoms, arthralgia, hepatic fibrosis, zinc deficiency and fluctuating levels of transaminases. 5,6 Several conditions can lead to lens deposits. Myotonic dystrophy and hypocalcaemia are associated with polychromatic iridescent deposits.…”

Agranulocytosis, arthritis and systemic vasculitis in a patient receiving the oral iron chelator L1 (deferiprone)

“…Alternatively, it has been hypothesized that agranulocytosis might be an idiosyncratic reaction against deferiprone or one of its metabolites (Hoffbrand, 1996). Arthropathy has been attributed to L1-induced shifts of iron from tissue stores to joint fluid and subsequent formation, by uncomplexed iron, of free radicals that peroxidate synovial membranes (Berkovitch et al, 1994). However, the demonstration of intra-articular deferiprone-iron complexes is lacking and the pathogenesis of arthropathy remains speculative.…”

“…Among these, agranulocytosis (4%), arthropathy (21%), liver enzyme abnormalities (44%) and severe nausea (8%) are the most important, sometimes requiring permanent discontinuation of treatment (Al-Refaie et al, 1995). Immunological abnormalities, including antinuclear antibodies (ANA) and antibodies to histones (AHA) positivity, seem to occur more frequently in thalassaemic patients treated with L1 than in those on conventional chelation therapy with desferrioxamine, but whether such abnormalities are relationed with L1-induced toxicity remains unknown (Mehta et al, 1991;Berkovitch et al, 1994).…”

Role of deferiprone in chelation therapy for transfusional iron overload

“…Some investigators have proposed chelator-induced transit of iron into the synovial space. 81 Others have related joint symptoms to higher levels of deferiprone or greater levels of iron overload. 11 The association of joint problems with higher serum ferritin levels found in the multicenter safety study after 1 year was not sustained after 4 years.…”