Primary digestive Kaposi sarcoma with idiopathic HIV-, HHV8+, CD4+ lymphocytopenia

A,52-year-old Tunisian patient had fever, impaired health and several oppor tunistic infections (Campylobacter jejuni, Mycobacterium hominis, Herpes vi rus, Giardia intestinalis, Vibrio metschnikovii). Lymphocytopenia was noted (348/mm(3); CD4+: 2.2%; CD4+/CD8+: 0.1). Polymerase chain rection search f or HIV was negative in serum and in tumor tissue. Diagnosis of primary dige stive Kaposi sarcoma was established at autopsy due to the deep location of the lesions. There was an ulcerofungating tumor spreading over 1.3 m of th e duodenojejunum. This is-the fourth reported case of CD4+ lymphocytopenia, a new and very rare immunodeficiency syndrome recently defined by the Cent ers for Disease Control. We detected human herpes virus 8 by immunohistoche mistry of tumor tissue. Human herpes virus 8 is implicated in the pathogene sis of Kaposi sarcoma.