Table of Contents
Definition / general | Diagrams / tables | Clinical features | Uses by pathologists | Case reports | Microscopic (histologic) images | Positive staining - normal | Positive staining - disease | Negative stainingCite this page: Pernick N. CD1d. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/cdmarkerscd1d.html. Accessed January 15th, 2021.
Definition / general
- CD1d presents lipid antigens to natural killer T (NKT) cells; when activated, NKT cells rapidly produce Th1 and Th2 cytokines, typically represented by interferon gamma and IL4 (Wikipedia: CD1D [Accessed 31 July 2018], OMIM: 188410 [Accessed 31 July 2018])
- B cells also present lipid antigen to CD1d restricted invariant NKT cells, which contributes to maintaining tolerance in autoimmunity
- On chromosome 1q22-23 (not MHC linked)
- Also called R3G1
- Sole group 2 member of the CD1 family of MHC-like glycoproteins
Diagrams / tables
Clinical features
- CD1d expression < 45% is predictive of CLL (Am J Clin Pathol 2011;136:400); downregulated by B-CLL (Leukemia 2002;16:2429), although the invariant natural killer T cell and CD1d axis is fundamentally intact in patients with early stage disease (Haematologica 2013;98:376)
- In paroxysmal nocturnal hemoglobinuria, a novel, autoreactive, CD1d restricted, glycosylphosphatidylinositol specific T cell population is expanded and may cause bone marrow failure (Blood 2013;121:2753)
- B cells are essential for iNKT cell expansion and activation in healthy donors, but fail to exert a similar effect in SLE patients (Immunity 2012;36:477, J Exp Med 2010;207:943)
- May protect against lipid antigen rich infectious microbes on human scalp (J Clin Pathol 2005;58:1278)
- Downregulated by HPV (J Virol 2010;84:11614) and some other viruses (Viruses 2012;4:2379), but not CMV (Biochem Biophys Res Commun 2011;413:616)
- Expressed in NKT cells active in autoimmune diabetes, tumor rejection and some microbial infections
Uses by pathologists
- No significant clinical use by pathologists
- May play a role in future vaccine development (Proc Natl Acad Sci U S A 2010;107:13010)
Case reports
- 6 year old boy with no apparent immunodeficiency who suffered severe life-threatening infection with varicella vaccine virus, with low invariant natural killer T cells and diminished CD1d expression (J Infect Dis 2011;204:1893)
Microscopic (histologic) images
Positive staining - normal
- Dendritic cells, intestinal epithelial cells, B cell subset, NK T cell subset (Immunol Lett 2005;100:42, J Immunol 2005;175:4416), low levels in thymus and monocytes
- Bronchial epithelium (PLoS One 2011;6:e22726)
- Scalp epidermis (basal and granular keratinocytes), pilosebaceous units and eccrine glands
- Testis: Leydig cell, Sertoli cells, spermatogonia; moderate expression in spermatocytes (Ultrastruct Pathol 2011;35:124)
Positive staining - disease
- Some B and T cell malignancies; keratinocytes in psoriasis
Negative staining
- Normal B cells