Idiopathic CD4 lymphocytopenia (ICL) is characterized by the absolute CD4 count <300 cells/ml or <20% of total T cells and negativity of HIV infection.
A retrospective analysis of 4 female and 2 male patients with ICL.
The patients were diagnosed aged 16-46 years. One patient was referred because of panniculitis; one suffered from frequent respiratory-tract infections and selective IgA deficiency, another had meningococcal meningitis in his history. In the remaining, the cause of investigation was an incidental observation of lymphocytopenia. During the follow-up, one patient suffered from fevers of unknown origin which disappeared after antibiotic treatment. However, no other manifestations of immunodeficiency were observed.
CD4+ cell numbers were between 33-190 cells/ml and were relatively stable during follow-up.
All patients had a decreased proportion of CD45RA+ cells in CD4+ cells. The proportion of gamma/delta T-lymphocytes in CD3+ cells was increased in 5 patients.
In 5 patients, a decrease in the absolute number of CD8+ cells was observed, NK cells absolute numbers were normal in 5 and increased in 1 patient.
The absolute number of B lymphocytes was decreased in 2 patients, normal in 4. Evaluating B-cell peripheral differentiation markers, variable results were observed.
Except for IgA deficiency in one patient and one transitory decrease in IgG and IgM, other major serum immunoglobulin levels were in the reference ranges. In 3 patients antinuclear antibodies were detected, another had antibodies against cardiolipin and thyroid peroxidase.
Our results show variable laboratory manifestation of ICL; clinical immunodeficiency symptoms are frequently missing.