idiopathic CD4 lymphocytopenia
A rare primary immunodeficiency disorder characterized by persistent CD4 T-cell lymphopenia (less than 300 cells/B5L on multiple occasions) not associated with any other underlying primary or secondary immune deficiency. Patients typically present opportunistic infections (with cryptococcal, mycobacterial, candidal, varicella zoster virus infections and progressive multifocal leukoencephalopathy being the most prevalent), malignancies (mainly lymphoproliferative disorders), or autoimmune disorders. Some individuals are asymptomatic and incidentally diagnosed.
MONDO:0018814
non-SCID combined immunodeficiency
excluded_subClassOf
MONDO:0021094
immunodeficiency disease
is_a
MONDO:0700007
idiopathic disease
is_a
ICL
related
IMD13
related
idiopathic CD4 positive T-lymphocytopenia
related
immunodeficiency 13
related
immunodeficiency type 13
exact