Seminari VHIR - Dr. Yenan Bryceson "Hemophagocytic Lymphohistiocytosis: signaling processes and activation of cytotoxic lymphocytes and NK"

Dr. Yenan Bryceson, Center for Hematology and Regenerative Medicine. Department of Medicine, Karolinska Institutet. Karolinska University Hospital - Stockholm- Sweden

"Hemophagocytic Lymphohistiocytosis: signaling processes and activation of cytotoxic lymphocytes and NK"

Defects in lymphocyte cytotoxicity are a cause of the life-threatening, early-onset hyperinflammatory syndrome hemophagocytic lymphohistiocytosis (HLH). Autosomal recessive mutations in PRF1, UNC13D, STXBP2, and STX11 have been associated with familial forms of HLH and disrupt lymphocyte-mediated target cell killing. A variety of other genetic mutations have also been associated with predisposition to HLH. I will review the molecular mechanism of lymphocyte cytotoxicity and efficacy of cellular assays for the diagnosis of patients. Moreover, the cellular basis of disease in different genetic forms of HLH will be discussed.

CV. Yenan Bryceson received his his PhD from Karolinska Institutet in 2008 after working in the lab of Eric Long at the NIH, and is currently Assistant Professor at the Center for Infectious Medicine at Karolinska University. His lab studies the molecular mechanisms of cytotoxic lymphocyte differentiation and function in the context of primary immunodeficiencies, hematological malignancies and systemic onset autoimmunity. The goal is to develop better diagnostic procedures and treatments for patients suffering rare genetic diseases, while providing further understanding of the human immune system that may also benefit the therapy of common diseases caused by dysregulated immunity. Yenan’s is recipient of an ERC starting grant and was recently named Wallenberg Academy Fellow. In his spare time he enjoys hiking, skiing, fly fishing and is trying to figure out surfing.

Host: Diagnostic Immunology Group, mmartinez@vhebron.net