HERPES-VIRUS (KSHV) ASSOCIATED KAPOSI-SARCOMA IN A 3-YEAR-OLD CHILD WITH NON-HIV-INDUCED IMMUNODEFICIENCY

A 3-year-old boy of German descent suffered from two episodes of Strep tococcus pneumoniae meningitis within 2 months. One month previously, the first skin lesion of Kaposi sarcoma (KS) had been observed behind his right ear. During the following 2 years KS disseminated not only m ucocutaneously but also to visceral organs. Immunological evaluation r evealed severe lymphocytopenia with reduced helper/suppressor T-cell r atio and impaired humoral immune response to pneumococci. Extensive la boratory tests gave no evidence for known immunocompromising infection s. However, recently described DNA sequences from a Kaposi sarcoma-ass ociated herpesvirus (KSHV) could be identified within skin tissue. As chemotherapy failed to stop tumour progression the patient was referre d for bone marrow transplantation. Eighteen months later the KS is in remission and the patient in a good general condition. Conclusion The case supports the hypothesis that KSHV is involved in the aetiology of KS. Bone marrow transplantation is possibly a therapeutic option for KS in patients with immunodeficiency not related to human immunodefici ency virus infection.