Myelodysplastic Syndrome (MDS) | Clinical Medicine

What's up ninja nerds? In this video today, we're going to be talking about milo displastic syndrome oftentimes referred to as MDS. So when we talk about myoisplastic syndrome, this is a disorder of the bone marrow. And I think one of the big things to remember is just kind of the basic pathophysiological concept behind this. When we think about this, it's really a mutation of the hematopoetic stem cells. And what I mean by this is that let's say that you take for example in the red bone marrow which is the site of hematopois right you have a hematopoetic stem cells if that hematopoetic stem cell develops mutations for some reason and it becomes abnormal let's say it becomes displastic that displastic stem cell is nasty in the sense that it can release cytoines and these cytoines have the ability to suppress the hematopitic stem cell from continuing to divide and differentiate into furler further myoid stem cells or lymphoid stem cells. Right? The issue with that is is that now if that hematopoitic stem cell under goes apoptosis that thing was supposed to go and make red cells and white cells and platelets. But if you kill it off now you lose the ability to make red cells, white cells and platelets and I mean normal ones. So because of that you can get a decrease in red blood cells, you can get a decrease in platelets and you can get a decrease in white cells. And this collectively the reduction in all cell lines is called panscytoenia. And it's usually an ominous sign that something's going on with the bone marrow potentially. Now the other sucky thing with MDS is that these displastic stem cells they try to take over as a normal hematopetic stem cells as ah I can do it and what it tries to do is it tries to make red blood cells but it's not very good and it has an ineffective is the terminology we say of making good red blood cells. So you get these like abnormal red blood cells that are usually very large and you don't make enough of them and so you get what's called a macroitic anemia which is a very common presentation of MDS. Another one is you may have a reduction in effective platelets which are supposed to help you get a clot. And so these patients can get a thrombocytoenia and lastly they may have ineffective lucopoesis and so they may have a low number of white blood cells especially neutrfils which can lead to neutropenia and again this contributes to that underlying panytoenia. So one of the big things with MDS is it may present with pansytoenia but oftent times don't ever discount a patient coming in with just macroitic anemia that it's not due to B12 or folate deficiency. And so that's really really important to remember. Another thing about these displastic stem cells is not only can they kill normal stem cells not only do they make less effective red cells, white cells and platelets, but they also can further mutate.