Fibrosis: Focus Area Two

By John D Crispino, PhD, Robert I. Lurie, MD and Lora S. Lurie Professor

I’m often asked to explain what exactly is bone marrow fibrosis. In non-technical terms, it is a condition in which the place where blood cell precursors live becomes unfriendly. You can think of the bone marrow niche as a comfortable bed, with a soft mattress covered by blankets and pillows. The blood stem cells live in this environment, which is warm and safe. However, when these stem cells and other cells in the bone marrow gain a mutation in JAK2, MPL, or CALR (among others that are yet to be identified), they are believed to produce substances that covert the comfortable bed into a bed of nails, with no pillows and no blankets. The blood cells no longer thrive in this environment and are forced to move out of the bone marrow and into other organs and the circulating blood. This contributes to the symptoms of myelofibrosis, including the reduction in blood counts.

Researchers are actively working to figure out how MPN mutations cause the changes in the bone marrow environment and to identify the specific types of mutant cells that are involved. Through its MPM Challenge, the MPN Research Foundation, in conjunction with the Leukemia and Lymphoma Society, are supporting numerous research groups in their efforts to learn more about this condition and to develop new therapies to reverse fibrosis.

Katya Ravid will be looking at MRI as an alternative to bone marrow biopsy for the detection of fibrosis. Leonard Zon is using zebrafish to decipher what the precursors are to the development of fibrosis. Since inflammation plays a role, Angela Fleischman’s project will look to understand more about how it contributes to fibrosis, looking specifically at a lymphoid cell responsible for inflammation. Lei Ding is looking at changes in the stem cells which are precursors to leukemia and MPNs. We’ll be updating you all with progress on these grants in the next year or so as their work progresses.

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