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Paroxysmal nocturnal haemoglobinuria phenotype cells and leucocyte subset telomere length in childhood acquired aplastic anaemia |
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| Authors: | Perri R. Tutelman Geraldine Aubert Ruth A. Milner Bakul I. Dalal Kirk R. Schultz Rebecca J. Deyell |
| Affiliation: | 1. Division of Pediatric Hematology/Oncology/Bone Marrow Transplantation, British Columbia Children's Hospital, University of British Columbia, , Vancouver, BC, Canada;2. Terry Fox Laboratory, British Columbia Cancer Agency, , Vancouver, BC, Canada;3. Child and Family Research Institute, University of British Columbia, , Vancouver, BC, Canada;4. Division of Laboratory Hematology, Department of Pathology and Laboratory Medicine, Vancouver General Hospital, , Vancouver, BC, Canada |
| 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. |
| Keywords: | aplastic anaemia children immunosuppressive therapy paroxysmal nocturnal haemoglobinuria phenotype cells leucocyte telomeres |