title: Retrospective nationwide survey of Japanese patients with transfusion-dependent MDS and aplastic anemia highlights the negative impact of iron overload on morbidity/mortality.
abstract: 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.
  
  
  METHODS
  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.
authors: Division of Hematology, Department of Medicine, Jichi Medical University, Tochigi, Japan. mtakatok@jichi.ac.jp
authors: Masaaki
authors: M
authors: Takatoku
authors: Takashi
authors: T
authors: Uchiyama
authors: Shinichiro
authors: S
authors: Okamoto
authors: Yuzuru
authors: Y
authors: Kanakura
authors: Kenichi
authors: K
authors: Sawada
authors: Masao
authors: M
authors: Tomonaga
authors: Shinji
authors: S
authors: Nakao
authors: Tatsutoshi
authors: T
authors: Nakahata
authors: Mine
authors: M
authors: Harada
authors: Takashi
authors: T
authors: Murate
authors: Keiya
authors: K
authors: Ozawa
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000741:Anemia, Aplastic / Q000378:metabolism* / Q000401:mortality; D001803:Blood Transfusion; D002423:Cause of Death; D003676:Deferoxamine / Q000627:therapeutic use; D005260:Female; D005293:Ferritins / Q000097:blood; D006801:Humans; D007501:Iron / Q000378:metabolism*; D007502:Iron Chelating Agents / Q000627:therapeutic use; D007564:Japan; D008297:Male; D008875:Middle Aged; D009190:Myelodysplastic Syndromes / Q000378:metabolism* / Q000401:mortality; D012189:Retrospective Studies
chemical_list: D007502:Iron Chelating Agents; D005293:Ferritins; D007501:Iron; D003676:Deferoxamine