期刊
JOURNAL OF PEDIATRICS
卷 144, 期 4, 页码 519-523出版社
MOSBY-ELSEVIER
DOI: 10.1016/j.jpeds.2003.11.012
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Objectives Mediterranean classic Kaposi sarcoma (KS) of childhood is rare and unexplained. Our objective is to describe the case of a child with complete IFNgammaRI1 deficiency and severe mycobacterial disease in whom Kaposi sarcoma (KS) developed. Results Disseminated myeobacterial 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-gamma-mediated immunity and severe mycobacterial disease may predispose HHV-8-infected children to KS.
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