Step I Myelodysplastic vs. Myelophthisic

[TIMMY]

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Can anyone help out with the difference betwen these 2....and how does a Myelodysplastic dz lead to a myelophthisic condition....or vice versa....
Blanking out on this! 😕

 

[catinahat]

Myelodysplastic Syndrome refers to a state (sometimes called pre-leukemia, but not so much anymore) where marrow failure and ineffective hematopoeisis predominate. There is an increased apoptotic rate of marrow cells and general stem cell depletion. Characteristic findings include ringed sideroblasts, megaloblastic maturation, messed up megakaryocytes, and bilobed PMNs. Progression to AML happens some of the time, and survival varies widely, from less than a year to more than 5. Those cases induced by therapy (radiation or drugs) are known as t-MDS and have an extremely poor prognosis. I think this might be because of patient age, though, as most patients are elderly and thus treated supportively, while younger patients receive BMT. I get the sense that it's not covered too much on boards, as neither BRS Path nor Goljan discusses it at all, and the Kaplan lecture notes have a total of three lines on it.

Myelophthistic anemia is a process in which metastatic cells or plain old fibrosis displace normal progenitors, causing marrow faliure. I think this too is considered low-yield, though it does get a note in BRS.

Thanks for making me think (and read). I hope this was helpful.

 

[BlackNDecker]

Perhaps a little more succint (and a little less Robbins-ish)...

It is easiest to divide myelogenous disorders in the following way:

Acute myelogenous leukemia - > 30% blasts...I believe this is still the cut off. Someone correct me if I'm wrong.

Myelodysplastic syndrome - clonal stem cell disorder...cells can't mature, however there are less than 20% blasts in the blood (therefore distinguishing it from AML)

Myelofibrosis - this is the end stage of the myeloproliferative disorders. There are 3 (CML, Polycythemia vera, essential thrombocytosis). What is important here is that you will see hepatomegaly and splenomegaly due to secondary hematopoiesis...thus it's alternate name Myelofibrosis with myeloid metaplasia (MMM)

 

[TIMMY]

Perhaps a little more succint (and a little less Robbins-ish)...

It is easiest to divide myelogenous disorders in the following way:

Acute myelogenous leukemia - > 30% blasts...I believe this is still the cut off. Someone correct me if I'm wrong.

Myelodysplastic syndrome - clonal stem cell disorder...cells can't mature, however there are less than 20% blasts in the blood (therefore distinguishing it from AML)

Myelofibrosis - this is the end stage of the myeloproliferative disorders. There are 3 (CML, Polycythemia vera, essential thrombocytosis). What is important here is that you will see hepatomegaly and splenomegaly due to secondary hematopoiesis...thus it's alternate name Myelofibrosis with myeloid metaplasia (MMM)

Exactly, Goljan, BRS or even robbins did not stress it but of course Q -bank had a question and one of the choices was Chronic Myelodysplastic Syndrome or leukemia...along those lines...the other options were out of the question in this case so I got the answer my elimination
Thanks for the help folks!

 

[BlackNDecker]

Exactly, Goljan, BRS or even robbins did not stress it

The hardest part is organizing it in your head so that it makes sense.

Sorry, I just noticed that you were also asking about myelophthisis...this is the change in marrow hematopoiesis caused by metastasis into the BM. Basically the tumor cells crowd out the BM stem cells rather than fibrosing due to BM exhaustion (myelodysplastic syndromes)

 

[Stressedoutdoc]

How about the difference between myelodysplastic and myeloproliferative disorder?....Thanks in advance

 

[tasar1898]

I guess myelodysplastic means your stem cells are trashed , so all non - lymphoid lineage will be dysplastic/immature.. The produced cells suck so much , that they just die--> cytopenias.
It's seen in old people and post chemo/radiation and can transform to AML

Myeloproliferative means that your stem cells are overworked and they produce tons of cells, that for the most part look/work normal , so you get excess of blood cells.. All are due to jak-stat gain of function mutation except CML--> 9,22 bcr-abl

 

[snowKNOWZ]

Myelodysplastic Syndrome refers to a state (sometimes called pre-leukemia, but not so much anymore) where marrow failure and ineffective hematopoeisis predominate. There is an increased apoptotic rate of marrow cells and general stem cell depletion. Characteristic findings include ringed sideroblasts, megaloblastic maturation, messed up megakaryocytes, and bilobed PMNs. Progression to AML happens some of the time, and survival varies widely, from less than a year to more than 5. Those cases induced by therapy (radiation or drugs) are known as t-MDS and have an extremely poor prognosis. I think this might be because of patient age, though, as most patients are elderly and thus treated supportively, while younger patients receive BMT. I get the sense that it's not covered too much on boards, as neither BRS Path nor Goljan discusses it at all, and the Kaplan lecture notes have a total of three lines on it.

Myelophthistic anemia is a process in which metastatic cells or plain old fibrosis displace normal progenitors, causing marrow faliure. I think this too is considered low-yield, though it does get a note in BRS.

Thanks for making me think (and read). I hope this was helpful.

Awesome answer. ⭐️⭐️⭐️⭐️⭐️ Aka “money”