[Idiopathic CD4 lymphocytopenia with lethal Salmonella typhimurium sepsis]

In April 1991, a then 43-year-old woman fell ill with a systemic cryptococcal infection which responded well to antimycotic treatment. Four months later she was found to have a T-helper cell deficiency (48/microliters, 15%). Since August 1992 she noted an increased tendency towards infections and had recurrent fever bouts over 4 weeks, which led to her hospitalization in January 1993. Repeated HIV tests were negative. During the first 4 days she was free of infection, but developed a temperature of 39.1 degrees C on the fifth day, and within a few hours a fulminant septicaemia with cardiorespiratory failure developed ending fatally the same day. Blood cultures drawn during fever bouts grew Salmonella typhimurium by the time of her death. Lymphocyte differentiation revealed absolute and relative reduction in the number of CD4-lymphocytes (158/microliters; 18%), retrospectively providing the diagnosis of idiopathic CD4-lymphocytopenia.--In cases such as this there is the need of including opportunistic and masked disease entities in the differential diagnosis, even in the absence of HIV infections, and start early treatment.