Idiopathic CD4 immunodeficiency is a rare disorder which may clinically resemble HIV disease.
Critiera for the diagnosis of CD4 lymphocytopenia:
(1) persistent CD4-positive T cell lymphocytopenia - one or both of the following:
(1a) <= 300 per µL
(1b) <= 20% of total lymphocytes
(2) exclusion of HIV-1 and HIV-2 infection
(3) exclusion of alternative explanation such as chemoradiation or immunosuppression
Persistent lymphocytopenia involves a minimum of two separate analysis at least 6 weeks apart.
Clinical features may include:
(1) opportunistic infections (cryptococcus, HPV, CMV, PML, pneumocystis, etc)
(2) autoimmune disorder
(3) malignant lymphoma
(4) solid tumor
Other findings may include an absolute NK cell lymphocytopenia. An absolute NK cell count < 100 per µL is associated with increased mortality.
