Journal of Leukocyte Biology
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Published online before print June 14, 2004
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© by The Society for Leukocyte Biology
Journal of Leukocyte Biology, doi:10.1189/jlb.1203607

Received for publication December 2, 2003.
Revised April 8, 2004.
Accepted for publication May 20, 2004.

Article

GPI-defective monocytes from paroxysmal nocturnal hemoglobinuria patients show impaired in vitro dendritic cell differentiation

Giuseppina Ruggiero *@, Giuseppe Terrazzano *, Cristina Becchimanzi {dagger}, Michela Sica *, Claudia Andretta {dagger}, Anna Maria Masci *, Luigi Racioppi *, Bruno Rotoli {dagger}, Serafino Zappacosta *, and Fiorella Alfinito {dagger}

Cattedra di *Immunologia, Dipartimento di Biologia e Patologia Cellulare e Molecolare, and {dagger}Ematologia, Dipartimento di Medicina Clinica e Sperimentale, Università Federico II, Naples, Italy

@ To whom correspondence should be addressed. E-mail: giruggie{at}unina.it.


  Abstract

Paroxysmal nocturnal hemoglobinuria (PNH) is a clonal, acquired hematopoietic disorder characterized by a phosphatidylinositol (PI) glycan-A gene mutation, which impairs the synthesis of the glycosyl-PI (GPI) anchor, thus causing the absence of all GPI-linked proteins on the membrane of the clonal-defective cells. The presence of a consistent GPI-defective monocyte compartment is a common feature in PNH patients. To investigate the functional behavior of this population, we analyzed its in vitro differentiation ability toward functional dendritic cells (DCs). Our data indicate that GPI-defective monocytes from PNH patients are unable to undergo full DC differentiation in vitro after granulocyte macrophage-colony stimulating factor and recombinant interleukin (IL)-4 treatment. In this context, the GPI-defective DC population shows mannose receptor expression, high levels of the CD86 molecule, and impaired CD1a up-regulation. The analysis of lipopolysaccharide and CD40-dependent, functional pathways in these DCs revealed a strong decrease in tumor necrosis factor {alpha} and IL-12 production. Finally, GPI-defective DCs showed a severe impairment in delivering accessory signals for T cell receptor-dependent T cell proliferation.




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