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Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a rare life‐threatening disease in which the immune system becomes overactive due to its inability to effectively respond to infections and/or shut down the immune response to such infections. The discovery of genetic defects in the secretory pathway of natural killer (NK) cells and cytotoxic T cells in some patients with this disease has raised important questions of the role of cytotoxic cells in the control of infections and in immune regulation. This review will give a brief overview of the clinical presentation and accepted treatment of HLH. Furthermore, it will give an in‐depth review into the known genetic defects and current knowledge of the pathophysiology of this disorder, and will highlight recent evidence suggesting that cytotoxic defects in CD4+ T regulatory cells may contribute to the pathogenesis of HLH.
| Abbreviations | ||
| IL | = | interleukin |
| IFN | = | interferon |
| TNF | = | tumor necrosis virus |
| ESR | = | erythrocyte sedimentation rate |
| CMV | = | cytomegalovirus |
| HHV | = | human herpes virus |
| VSV | = | Varicella zoster virus |
| TGF | = | transforming growth factor |
| GTP | = | guanosine triphosphate |
| Ras | = | rat sarcoma oncogene |
| PBMC | = | peripheral blood mononuclear cell |
| HSV | = | herpes sinplex virus |
| Abbreviations | ||
| IL | = | interleukin |
| IFN | = | interferon |
| TNF | = | tumor necrosis virus |
| ESR | = | erythrocyte sedimentation rate |
| CMV | = | cytomegalovirus |
| HHV | = | human herpes virus |
| VSV | = | Varicella zoster virus |
| TGF | = | transforming growth factor |
| GTP | = | guanosine triphosphate |
| Ras | = | rat sarcoma oncogene |
| PBMC | = | peripheral blood mononuclear cell |
| HSV | = | herpes sinplex virus |
Acknowledgements
This work was supported by the Advancing a Healthier Wisconsin program (WJG), the Hope Street Kids (WJG), and the Abbott Scholars Award in Rheumatology Research (JWV). We thank David Wilson for his critical review of this manuscript.