Selective deficiency of naïve CD4+ T-lymphocytes in a child with congenital lymphoedema.

We describe the first known case of congenital lymphoedema associated with selective deficit of naïve CD4+ T-lymphocytes. A high proportion of naïve CD4+ T-lymphocytes was found in the ascitic fluid, supporting the hypothesis of extra-vascular sequestration of these cells into lymphoedematous tissue.