[Primary digestive tract Kaposi sarcoma with idiopathic CD4+ lymphocytopenia, HIV negative, HHV8 positive]

A 52-year-old Tunisian patient had fever, impaired health and several opportunistic infections (Campylobacter jejuni, Mycobacterium hominis, Herpes virus, Giardia intestinalis, Vibrio metschnikovii). Lymphocytopenia was noted (348/mm3; CD4+: 2.2%; CD4+/CD8+: 0.1). Polymerase chain rection search for HIV was negative in serum and in tumor tissue. Diagnosis of primary digestive 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 the duodenojejunum. This is the fourth reported case of CD4+ lymphocytopenia, a new and very rare immunodeficiency syndrome recently defined by the Centers for Disease Control. We detected human herpes virus 8 by immunohistochemistry of tumor tissue. Human herpes virus 8 is implicated in the pathogenesis of Kaposi sarcoma.