HHV-8–associated Kaposi sarcoma in a child with IFNγR1 deficiency - 24/08/11
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, Capucine Picard, MD , Vincent Lacoste, PhD, Stéphanie Dupuis, PhD, Necla Akçakaya, MD, Haluk Çokura, MD, Gültekin Kaner, MD, Cuyan Demirkesen, MD, Sabine Plancoulaine, MD, PhD, Jean-François Emile, MD, PhD, Antoine Gessain, MD, PhD, Jean-Laurent Casanova, MD, PhD ⁎ Yildiz Camcioglu, Department of Pediatrics, Infectious Diseases and Clinical Immunology Division, Cerrahpasa Medical School, University of Istanbul, Istanbul, Turkey.Reprint requests: Jean Laurent Casanova, Laboratoire de Génétique Humaine des Maladies Infectieuses, Université René Descartes, INSERM U550, Faculté de Médecine Necker, 156 Rue de Vaugirard, 75015 Paris, France.See related articles p 421 p 512 and p 524.
Abstract |
Objectives |
Mediterranean classic Kaposi sarcoma (KS) of childhood is rare and unexplained. Our objective is to describe the case of a child with complete IFNγR1 deficiency and severe mycobacterial disease in whom Kaposi sarcoma (KS) developed.
Results |
Disseminated mycobacterial infection began at the age of 5 months, and at 11 years of age the child had disseminated KS lesions. The histologic appearance of these lesions was typical, with endothelial and spindle cell proliferation. Human herpesvirus-8 (HHV-8)-associated antigens were detected in situ by immunohistochemistry. HHV-8 DNA of K1 molecular subtype A was amplified from tissue lesions, and HHV-8–specific antibodies were detected in the patient's serum. The child died at 12 years of age of disseminated mycobacterial disease and KS.
Conclusions |
This is the first identification of a well-defined primary immunodeficiency in a child with KS. Inherited disorders of IFN-γ–mediated immunity and severe mycobacterial disease may predispose HHV-8–infected children to KS.
Le texte complet de cet article est disponible en PDF.Abbreviations : BCG, HHV-8, HIV, IFN-⍺, KS
Plan
 | Supported by the Fondation BNP-Paribas, the Fondation Schlumberger, and INSERM (C. P. QLK2-CT-2002-00846). |
Vol 144 - N° 4
P. 519-523 - avril 2004 Retour au numéro
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