Paroxysmal nocturnal haemoglobinuria phenotype cells and leucocyte subset telomere length in childhood acquired aplastic anaemia.
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.
Division of Pediatric Hematology/Oncology/Bone Marrow Transplantation, British Columbia Children's Hospital, University of British Columbia, Vancouver, BC, Canada.
Perri R
PR
Tutelman
Geraldine
G
Aubert
Ruth A
RA
Milner
Bakul I
BI
Dalal
Kirk R
KR
Schultz
Rebecca J
RJ
Deyell
D000293:Adolescent; D000741:Anemia, Aplastic / Q000150:complications* / Q000188:drug therapy / Q000235:genetics / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006457:Hemoglobinuria, Paroxysmal / Q000150:complications* / Q000235:genetics / Q000276:immunology; D006801:Humans; D016130:Immunophenotyping; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007223:Infant; D007962:Leukocytes / Q000648:ultrastructure*; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D016615:Telomere / Q000648:ultrastructure*; D059505:Telomere Homeostasis; D016896:Treatment Outcome
D007166:Immunosuppressive Agents
aplastic anaemia; children; immunosuppressive therapy; leucocyte telomeres; paroxysmal nocturnal haemoglobinuria phenotype cells