Abstract
The significance of paroxysmal nocturnal haemoglobinuria (PNH(pos) ) 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 PNH(pos) , of whom 94% achieved at least a partial response (PR) to IST; 11/16 (69%) achieved complete response (CR). Only 2/6 (33%) PNH(neg) patients achieved PR. PNH(pos) patients were less likely to fail IST compared to PNH(neg) 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.
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