Reconstitution of lymphocyte subpopulations in children with inherited metabolic storage diseases after haematopoietic cell transplantation

Paola Corti, Charles Peters, Adriana Balduzzi, Barbara Bertagnolio, Andrea Biondi, Cristina Bugarin, Maria Dassi, Francesca Furlan, Giuseppe Gaipa, Daniela Longoni, Oscar Maglia, Rossella Parini, Paolo Perseghin, Cornelio Uderzo, Graziella Uziel, Giuseppe Masera, Attilio Rovelli

Research output: Contribution to journalArticlepeer-review


We prospectively evaluated the reconstitution of lymphocyte subpopulations in nine children with lysosomal diseases who underwent 11 allogeneic haematopoietic cell transplants (HCTs) following CD34+ immunomagnetic enrichment, limited T-cell addback and in vivo B-cell depletion. Absolute lymphocyte count recovery was slow to cross the 5th percentile, occurring at a median of 10 months after HCT in patients with full chimaerism. Natural killer cells represented up to 90% of the total lymphoid population during the first 3 months. CD4+ lymphocyte recovery occurred 9-18 months after HCT. In most patients, CD8+ lymphocyte recovery was slow and comparable with that of CD4+ lymphocytes. The CD4+/CD8+ ratio normalised by 3-7 months after HCT in 50% of the patients. CD8+ lymphocyte recovery was enhanced in patients with viral reactivation. Reconstitution of B-lymphocytes was particularly delayed in patients treated with rituximab. Declining chimaerism, rejection and viral reactivation were the most common problems in our series. Because of the unique graft manipulation, the pace of lymphocyte reconstitution was particularly slow, suggesting that these patients are at a significantly increased risk of infections for up to 2 years after HCT.

Original languageEnglish
Pages (from-to)249-255
Number of pages7
JournalBritish Journal of Haematology
Issue number2
Publication statusPublished - Jul 2005


  • Haematopoietic cell transplantation
  • Immune reconstitution
  • Lysosomal diseases
  • Mucopolysaccharidosis
  • T-cell depletion

ASJC Scopus subject areas

  • Hematology


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