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Severe cutaneous papillomavirus disease after haemopoietic stem-cell transplantation in patients with severe combined immune deficiency caused by common γc cytokine receptor subunit or JAK-3 deficiency

2004; Elsevier BV; Volume: 363; Issue: 9426 Linguagem: Inglês

10.1016/s0140-6736(04)16457-x

1474-547X

Caroline Laffort, Françoise Le Deist, Michel Fãvre, Sophie Caillat‐Zucman, Isabelle Radford‐Weiss, Sylvie Fraitag, Stéphane Blanche, Marina Cavazzana, Geneviève de Saint Basile, Jean‐Pierre de Villartay, Silvia Giliani, Gérard Orth, Jean‐Laurent Casanova, Christine Bodemer, ALAIN FISCHER, Marianne Debré,

Oral Health Pathology and Treatment

Haemopoietic stem-cell transplantation is a life-saving treatment for severe combined immune deficiency. However, there has been little long-term follow-up of this treatment. There is evidence for the persistance of partial immunodeficiency associated with significant infections, including severe human papillomavirus (HPV) disease. We did a retrospective analysis of severe HPV disease in a group of 41 patients with severe combined immune deficiency from one centre who were alive 10 years or longer after haemopoietic stem-cell transplantation. Nine of the 41 patients had extensive chronic HPV disease limited to the skin, with a median onset at 8 years after transplantation. Four had lesions typical of epidermodysplasia verruciformis, a rare genodermatosis. Transplant characteristics, immune status, and chimerism of these nine patients did not differ significantly from those of the other patients. The nine patients with HPV disease had severe combined immune deficiency associated with either common γc receptor cytokine subunit or Janus kinase-3 (JAK-3) deficiency. By contrast, patients with other forms of severe combined immune deficiency did not have any signs of HPV disease. That genetic causes are the only predisposing factor to be identified for severe combined immune deficiency, suggests that natural-killer cells or γc/JAK-3-dependent signalling in keratinocytes could have a role in anti-HPV immunity.