October 7 2019
We would like to inform you that the 58th IRCMS Seminar has been scheduled as below. We would be pleased to see many of you participating in the seminar.
Date : October 15, 2019 (Tuesday)
Time : 16:00-17:00
Venue : IRCMS 1F Meeting Lounge
Speaker : Marc H.G.P. Raaijmakers, M.D., Ph.D.
Department of Hematology
Erasmus Medical Center Cancer Institute
Tilte :Hematopoietic stem cell responses to bone marrow failure
Elucidation of the molecular signaling underlying bone marrow failure and clonal evolution holds great promise for novel avenues to attenuate leukemic evolution in pre-leukemic syndromes. Congenital, monogenic, bone marrow failure syndromes with leukemia predisposition, in which mutations in a single gene, present in both HSPC and niche cells, drives tissue failure, clonal evolution and malignant transformation, provide an unprecedented model system to study the molecular mechanisms and human relevance of these concepts. The lecture will address the findings of studies in Shwachman-Diamond Syndrome (SDS), caused by constitutive, bi-allelic, loss-of-function, mutations in the ribosome biogenesis gene SBDS, and characterized by bone abnormalities, neutropenia and a striking propensity to develop MDS/AML. Hematopoietic cell autonomous and niche contributions will be discussed and emerging data on the effect of cytopenia on stem cell function presented.