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著者: Masaaki Takatoku, Takashi Uchiyama, Shinichiro Okamoto, Yuzuru Kanakura, Kenichi Sawada, Masao Tomonaga, Shinji Nakao, Tatsutoshi Nakahata, Mine Harada, Takashi Murate, Keiya Ozawa, Japanese National Research Group on Idiopathic Bone Marrow Failure Syndromes
雑誌名: Eur J Haematol. 2007 Jun;78(6):487-94. doi: 10.1111/j.1600-0609.2007.00842.x. Epub 2007 Mar 28.
Abstract/Text
OBJECTIVE: Myelodysplastic syndromes (MDS) and aplastic anemia (AA) are the most common anemias that require transfusion therapy in Japan. This retrospective survey investigated relationships between iron overload, chelation practices, and morbidity/mortality in patients with these diseases.
METHOD: Medical histories of transfusion-dependent patients were assessed at transfusion onset, chelation onset, and study end.
RESULTS: Data were collected from 292 patients with MDS, AA, pure red cell aplasia, myelofibrosis, and other conditions. Patients received a mean of 61.5 red blood cell units during the previous year. Fewer than half (43%) of patients had previously received deferoxamine (DFO) therapy. Only 8.6% received daily/continuous DFO. In all, 75 deaths were reported, with cardiac and liver failure noted in 24.0 and 6.7% of cases. Of these, 97% had ferritin levels >1000 ng/mL. Abnormal cardiac and liver function was observed in 21.9% (14/64) and 84.6% (11/13) of all patients assessed. Effective chelation with DFO resulted in improved serum ferritin, liver enzymes, and fasting blood sugar.
CONCLUSIONS: Mortality is higher in heavily iron-overloaded patients, with liver and cardiac dysfunction being the primary cause. Daily/continuous chelation therapy was effective at reducing iron burden and improving organ function. Chelation therapy should be initiated once serum ferritin levels exceed 1000 ng/mL.
PMID 17391310 Eur J Haematol. 2007 Jun;78(6):487-94. doi: 10.1111/j.1600-0609.2007.00842.x. Epub 2007 Mar 28.
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