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Retrospective nationwide survey of Japanese patients with transfusion‐dependent MDS and aplastic anemia highlights the negative impact of iron overload on morbidity/mortality
Author(s) -
Takatoku Masaaki,
Uchiyama Takashi,
Okamoto Shinichiro,
Kanakura Yuzuru,
Sawada Kenichi,
Tomonaga Masao,
Nakao Shinji,
Nakahata Tatsutoshi,
Harada Mine,
Murate Takashi,
Ozawa Keiya
Publication year - 2007
Publication title -
european journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0902-4441
DOI - 10.1111/j.1600-0609.2007.00842.x
Subject(s) - medicine , deferoxamine , chelation therapy , myelodysplastic syndromes , aplastic anemia , blood transfusion , deferasirox , gastroenterology , myelofibrosis , anemia , liver function , ferritin , transfusion therapy , pediatrics , surgery , thalassemia , bone marrow
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.