In early July, we reported The MPN Research Foundation and LLS granted $1 million research funding for MPNs. Dr Angela Fleischman has been a key contributor to PV Reporter and was one of the MPN Challenge award winners. I asked her for a description of the research they will be working on and she provided the summary below.
Areas of research include the prominently discussed Excessive Inflammation in MPN, Mutated Calreticulin in MPN and the Role of Lymphocytes in MPN Pathogenesis.
Dr Fleischman suggests:
“Inflammation is not only responsible for the clinical consequences of MPN but it is also critical for disease initiation.”
“This excessive inflammatory state is responsible for the debilitating constitutional symptoms frequently observed in this disease.”
MPN Challenge 2014 Award – Dr. Angela Fleischman and Dr. Rick Van Etten
by Dr. Angela Fleischman
Inflammatory proteins (also known as cytokines) are elevated in the blood of MPN patients and not only contribute to the symptoms associated with the disease but may also play a key role in MPN disease initiation and maintenance. The immune system is composed of many types of cells which serve to fight infection and heal wounds. Abnormal regulation of immune cells leads to consequences such as chronic inflammation.
We have found that lymphocytes, a class of immune cells implicated in many autoimmune diseases, are also important for development of MPN in a model system.
Lymphocytes from MPN patients only rarely have the JAK2V617F mutation, so we propose that the culprit lymphocytes reacting to the presence of JAK2V617F cells in the body rather than a direct consequence of JAK2V617F in the lymphocytes themselves. We will determine whether lymphocytes from MPN patients react abnormally to cells bearing the JAK2V617F mutation and if so identify why they do so. We will use lymphocytes from peripheral blood of MPN patients for these studies. We will also identify the specific lymphocyte cell type that contributes to MPN pathology with the purpose of developing MPN therapies targeting this cell.