Dendritic cells are antigen-presenting cells that connect innate and adaptive immunity by recognizing and presenting antigen to T cells. Although the relevance of dendritic cells has been shown in several animal models, their role in human immunity remains largely unknown in vivo. Dendritic cell loss or malfunction would be anticipated to have clinical implications given their prominent involvement in infection, autoimmunity, and cancer. Human dendritic cell deficiency disorders caused by mutations in GATA binding protein 2 (GATA2) and interferon regulatory factor 8 (IRF8) have highlighted the importance of dendritic cells and monocytes in primary immunodeficiency diseases and focuses on their nonredundant roles in host defense and immune regulation in vivo. It is becoming clear that dendritic cell and monocyte malfunction have a role in the development of primary immunodeficiency disease patterns. Dendritic cell analysis, on the other hand, is not yet a standard element of the workup for primary immunodeficiency illness.

The widespread use of dendritic cell/monocyte screening in clinical practice will aid in the detection of new dendritic cell and monocyte diseases while also furthering our understanding of human dendritic cell biology in health and illness.