Abstract:
:Transfusion is believed to be the main cause of iron overload in Japan. A nationwide survey on post-transfusional iron overload subsequently led to the establishment of guidelines for iron chelation therapy in this country. To date, however, detailed clinical information on the entire iron overload population in Japan has not been fully investigated. In the present study, we obtained and studied detailed clinical information on the iron overload patient population in Japan. Of 1109 iron overload cases, 93.1% were considered to have occurred post-transfusion. There were, however, 76 cases of iron overload of unknown origin, which suggest that many clinicians in Japan may encounter some difficulty in correctly diagnosing and treating iron overload. Further clinical data were obtained for 32 cases of iron overload of unknown origin; median of serum ferritin was 1860.5 ng/mL. As occurs in post-transfusional iron overload, liver dysfunction was found to be as high as 95.7% when serum ferritin levels exceeded 1000 ng/mL in these patients. Gene mutation analysis of the iron metabolism-related genes in 27 cases of iron overload with unknown etiology revealed mutations in the gene coding hemojuvelin, transferrin receptor 2, and ferroportin; this indicates that although rare, hereditary hemochromatosis does occur in Japan.
journal_name
Int J Hematoljournal_title
International journal of hematologyauthors
Ikuta K,Hatayama M,Addo L,Toki Y,Sasaki K,Tatsumi Y,Hattori A,Kato A,Kato K,Hayashi H,Suzuki T,Kobune M,Tsutsui M,Gotoh A,Aota Y,Matsuura M,Hamada Y,Tokuda T,Komatsu N,Kohgo Ydoi
10.1007/s12185-016-2141-9subject
Has Abstractpub_date
2017-03-01 00:00:00pages
353-360issue
3eissn
0925-5710issn
1865-3774pii
10.1007/s12185-016-2141-9journal_volume
105pub_type
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