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- Title
Paroxysmal nocturnal haemoglobinuria phenotype cells and leucocyte subset telomere length in childhood acquired aplastic anaemia.
- Authors
Tutelman, Perri R.; Aubert, Geraldine; Milner, Ruth A.; Dalal, Bakul I.; Schultz, Kirk R.; Deyell, Rebecca J.
- Abstract
The significance of paroxysmal nocturnal haemoglobinuria ( PNHpos) cells and leucocyte subset telomere lengths in paediatric aplastic anaemia ( AA) is unknown. Among 22 children receiving immunosuppressive therapy ( IST) for AA, 73% (16/22) were PNHpos, of whom 94% achieved at least a partial response ( PR) to IST; 11/16 (69%) achieved complete response ( CR). Only 2/6 (33%) PNHneg patients achieved PR. PNHpos patients were less likely to fail IST compared to PNHneg patients (odds ratio 0·033; 95% confidence interval 0·002-0·468; P = 0·012). Children with AA had short granulocyte ( P = 7·8 × 10−9), natural killer cell ( P = 6·0 × 10−4), naïve T lymphocyte ( P = 0·002) and B lymphocyte ( P = 0·005) telomeres compared to age-matched normative data.
- Subjects
PAROXYSMAL hemoglobinuria; ANEMIA; TELOMERES; LEUCOCYTES; GRANULOCYTES; KILLER cells; IMMUNOSUPPRESSION; LYMPHOCYTES; THERAPEUTICS
- Publication
British Journal of Haematology, 2014, Vol 164, Issue 5, p717
- ISSN
0007-1048
- Publication type
Article
- DOI
10.1111/bjh.12656