British Journal of Haematology
British Journal of Haematology
5 years ago

Development of clinical paroxysmal nocturnal haemoglobinuria in children with aplastic anaemia

Atsushi Narita, Kyogo Suzuki, Daisuke Ichikawa, Yoshiyuki Takahashi, Eri Nishikawa, Seiji Kojima, Hideki Muramatsu, Nobuhiro Nishio, Rieko Taniguchi, Nozomu Kawashima, Daiei Kojima, Shinsuke Kataoka, Norihiro Murakami, Yuko Sekiya, Motoharu Hamada, Yusuke Okuno, Asahito Hama
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.

Publisher URL: http://onlinelibrary.wiley.com/resolve/doi

DOI: 10.1111/bjh.14790

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