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Originally published online as doi:10.1189/jlb.1203607 on June 14, 2004

Published online before print June 14, 2004
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(Journal of Leukocyte Biology. 2004;76:634-640.)
© 2004 by Society for Leukocyte Biology

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

Giuseppina Ruggiero*,1, 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

1 Correspondence: Cattedra di Immunologia-Dipartimento di Biologia e Patologia Cellulare e Molecolare, Università di Napoli "Federico II", Via Pansini 5, 80131 Naples, Italy. E-mail: giruggie{at}unina.it

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.

Key Words: GPI-linked molecules • cytokine secretion • accessory signals




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