Paroxysmal nocturnal haemoglobinuria phenotype cells and leucocyte subset telomere length in childhood acquired aplastic anaemia

Br J Haematol. 2014 Mar;164(5):717-21. doi: 10.1111/bjh.12656. Epub 2013 Nov 14.

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.

Keywords: aplastic anaemia; children; immunosuppressive therapy; leucocyte telomeres; paroxysmal nocturnal haemoglobinuria phenotype cells.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Anemia, Aplastic / complications*
  • Anemia, Aplastic / drug therapy
  • Anemia, Aplastic / genetics
  • Anemia, Aplastic / immunology
  • Child
  • Child, Preschool
  • Female
  • Hemoglobinuria, Paroxysmal / complications*
  • Hemoglobinuria, Paroxysmal / genetics
  • Hemoglobinuria, Paroxysmal / immunology
  • Humans
  • Immunophenotyping
  • Immunosuppressive Agents / therapeutic use
  • Infant
  • Leukocytes / ultrastructure*
  • Male
  • Prognosis
  • Retrospective Studies
  • Telomere / ultrastructure*
  • Telomere Homeostasis
  • Treatment Outcome

Substances

  • Immunosuppressive Agents