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Role of innate immune signaling in myelodysplastic syndromes

Linda Ya-Ting Chang

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Canadian Institutes of Health Research (CIHR)
Myelodysplastic syndromes (MDS) are a group of blood-related diseases originating from disorder of the stem cells in the bone marrow, where all blood cells are formed. MDS are characterized by ineffective production of the myeloid-type blood cells, leading to anemia and loss of other normal blood cells. Patients with MDS often progress to bone marrow failure, showing extremely low blood count, or acute myeloid leukemia (AML), which has very poor prognosis. The only curative treatment for MDS is stem cell transplant, which is risky and not an option for the elderly MDS patients. Current treatments in MDS focus on improving the quality of life, decreasing the need for frequent blood transfusion, and decreasing the frequency of progression to AML. There is a lack of understanding in the cause of MDS and the subsequent AML transformation. Our lab has previously shown that the signals utilized by the myeloid white blood cells in the event of a microbial infection (the innate immune signaling pathway) are increased in the blood stem cells of a subset of MDS patients. Our lab generated a mouse model where innate immune signal in transplanted blood cells is amplified by increasing expression of one of the component proteins, TIRAP. The mice developed either bone marrow failure or AML, similar to MDS patients. By studying how TIRAP is inducing the hematological malignancies in the mouse model, we can further understand the mechanism of disease progression in MDS. Finally, using a genomics approach and the expertise of the Genome Sciences Centre, we can correlate our findings with available MDS/AML patient materials. Current research can then be extended to the development of drugs targeting specifically MDS stem cells. Ultimately, a more direct and targeted treatment plan for MDS patients with increased innate immune signaling can be generated from this research. The prevention of bone marrow failure or AML progression will improve patients' prognosis and quality of life.

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