| Literature DB >> 28643364 |
Atsushi Narita1, Hideki Muramatsu1, Yusuke Okuno1, Yuko Sekiya1, Kyogo Suzuki1, Motoharu Hamada1, Shinsuke Kataoka1, Daisuke Ichikawa1, Rieko Taniguchi1, Norihiro Murakami1, Daiei Kojima1, Eri Nishikawa1, Nozomu Kawashima1, Nobuhiro Nishio1, Asahito Hama1, Yoshiyuki Takahashi1, Seiji Kojima1.
Abstract
The clinical significance of paroxysmal nocturnal haemoglobinuria (PNH) in children with aplastic anaemia (AA) remains unclear. We retrospectively studied 57 children with AA between 1992 and 2010. During the follow-up, five patients developed clinical PNH, in whom somatic PIGA mutations were detected by targeted sequencing. The 10-year probability of clinical PNH development was 10·2% (95% confidence interval, 3·6-20·7%). Furthermore, the detection of minor PNH clones by flow cytometry at AA diagnosis was a risk factor for the subsequent development of clinical PNH. These patients with PNH clones at AA diagnosis should undergo periodic monitoring for potential clinical PNH development.Entities:
Keywords: zzm321990zzm321990PIGAzzm321990zzm321990; Children; aplastic anaemia; paroxysmal nocturnal haemoglobinuria
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Year: 2017 PMID： 28643364 DOI： 10.1111/bjh.14790
Source DB: PubMed Journal: Br J Haematol ISSN： 0007-1048 Impact factor: 6.998