Glossary of 2. Myelodysplastic Syndromes

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what is a stem cell?
a Pluripotent CFU-Blast that has the capacity to continuously self-replicate.
what does the CFU blast differentiate into?
CFU-GEMM or lymphoid stem cell
what does the CFU-GEMM diff into?
BFU-E, Meg, GM, Eos, or Baso
What do the BFUs diff into?
What does the CFU-GM diff into?
CFU-Myeloblast or
What 3 things does the Lymphoid Stem cell diff into?
Pre-T cell
Pre-B cell
maybe an NK cell
What are the 5 "series" that categorize cells in the BM morphologically?
1. Erythroid series
2. Lymphoid series
3. Megakaryocytic series
4. Monocytic series
5. Granulocytic series
what are CSFs?
Colony stimulating factors - which are hematopoietic growth factors
what are the 2 pluripotent GFs?
1. Interleukin 1, 3, and 6
What are the 3 Lineage-restricted GFs?
1. G-CSF
2. M-CSF
3. Erythropoietin
what IS MDS?
what's another common name for it?
Myelodysplastic Syndrome
aka, Pre-leukemia
What is the problem in MDS, in a nutshell?
There's a clonal problem with the cells due to a stem cell problem, so development is off.
What is the formal definition of MDS?
A heterogenous group of disorders due to proliferation of abnormal stem cell; has dyspoeisis, progressive cytopenias of one/more cell lines.
what are 8 presenting features of MDS?
1. Men have more than women
2. Occurs people > 40 yrs old
3. Anemia
4. Neutropenia
5. Thrombocytopenia
6. Abnormal chromosomal karytp
7. N/incr cellularity
8. megaloblast/pelgerOID
How fast does MDS progress?
not very; insidiously
What are 2 results of the neutro/thrombocytopenias?
-Infection prone
-Bleeding tendencies
What morphologic changes in RBCs are seen in MDS?
What metabolic changes in RBCs are seen in MDS?
-changes in Ag and enzymes
-Acquired Hb H
-Increased Hb F
What morphologic changes indicate dysgranulopoeisis?
-Pelgeroid, but only 30%
-Mixed eos/basos granules
what metabolic chanes occur in the granulocytes?
-Chemotactic response
-Bacteriocidal activity
What morphologic changes are seen in platelets in MDS?
-Giant platelets
-Abnormal granulation
-Circltng micromegakaryocytes
-Hyper OR hypolobed
what's the NUMBERS GAME?
Used to classify leukemias:
(all nucleated cells = ANC)
How does the numbers game work?
1. If <50% ANC are nRBC, look at step 2. 2. If >30% ANC are blasts, AML

If <or=30% ANC are blasts, MDS

-20 instead of 30 for WHO
What are 5 FAB classifications of Myelodysplastic Syndromes?
1. Refractory Anemia RA
2. RA + Ringed Sideroblasts RARA
3. RA + Excess Blasts RAEB
4. Chronic Myelomonocytic Leukemia CMML
5. RA + Excess blasts in Transition RAEB-t
What are the 4 WHO MDS classes?
1. RA w/ or w/out RSideroblasts
2. Refractory Cytopenia w/ excess blasts (raeb)
3. 5q- syndrome
4. Idiopathic MDS
Which FAB MDS is not recognized as a syndrome by WHO, but as a disease?
CMML - chronic myelomonocytic leukemia
Which FAB MDS is not even recognized as an Myelodysp. syndrome or disease by WHO? What is it called?
Refractory Anemia with Excess Blasts in transition (RAEB-t)
-They say it's overt acute leukemia b/c >20% blasts in BM
what is the criteria for calling it Acute Leukemia?
>20% BM blasts for WHO
>30% BM blasts for FAB
What is the MDS scoring system for?
Determining the life expectancy!
What are some Negative MDS factors?
-Chromosomal abnormalities
-Defective CFU-GM in BM culture
-History or radiation/chemo
-CD34 cell markers present
-Abnormal pockets of immature precursors in BM
What are 5 therapies for MDS?
1. Support - blood transf, platelets, prophylactic antibiot
2. Vitamins/hormones
3. Chemo
4. Biologic response modifiers
5. Bone marrow transplant (not very optimistic tho)

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