Clinical impact of HLA-DR15, a minor population of paroxysmal nocturnal haemoglobinuria-type cells, and an aplastic anaemia-associated autoantibody in children with acquired aplastic anaemia

Nao Yoshida, Hiroshi Yagasaki, Yoshiyuki Takahashi, Tomoko Yamamoto, Juan Liang, Yue Wang, Makito Tanaka, Asahito Hama, Nobuhiro Nishio, Ryoji Kobayashi, Noriko Hotta, Keiko Asami, Atsushi Kikuta, Takashi Fukushima, Naoto Hirano, Seiji Kojima

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Abstract

Aplastic anaemia (AA) is defined as a pancytopenia caused by bone marrow failure, and its pathogenesis is thought to involve autoimmune processes. Several predictive markers of the response to immunosuppressive therapy (IST) have been proposed, which appear to reflect the immune pathophysiology. We prospectively investigated the presence of human leucocyte antigen (HLA)-DR15, a minor population of paroxysmal nocturnal haemoglobinuria (PNH)-type cells, and antibodies to the recently identified autoantigen postmeiotic segregation increased 1 (PMS1) in 103 children with AA enrolled in a multicentre study. In contrast to adults, children with AA did not show an increased frequency of HLA-DR15. In addition, a sensitive flow cytometric assay revealed that children with AA have a much lower prevalence of PNH-type cells (21.4%) than reported for adults with this disease. An immunoblotting assay detected anti-PMS1 antibody in 15 of 103 (14.6%) of the children. Finally, the response rate to IST was not significantly different between patients with and without DR15 (45.5% vs. 54.0%), PNH-type cells (68.2% vs. 53.1%) or anti-PMS1 antibody (40.0% vs. 59.1%). The current study did not confirm a correlation between these markers and the response to IST, suggesting that there is a difference in the pathophysiologies of adult and paediatric AA.

Original languageEnglish
Pages (from-to)427-435
Number of pages9
JournalBritish Journal of Haematology
Volume142
Issue number3
DOIs
Publication statusPublished - 08-2008

All Science Journal Classification (ASJC) codes

  • Hematology

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    Yoshida, N., Yagasaki, H., Takahashi, Y., Yamamoto, T., Liang, J., Wang, Y., Tanaka, M., Hama, A., Nishio, N., Kobayashi, R., Hotta, N., Asami, K., Kikuta, A., Fukushima, T., Hirano, N., & Kojima, S. (2008). Clinical impact of HLA-DR15, a minor population of paroxysmal nocturnal haemoglobinuria-type cells, and an aplastic anaemia-associated autoantibody in children with acquired aplastic anaemia. British Journal of Haematology, 142(3), 427-435. https://doi.org/10.1111/j.1365-2141.2008.07182.x