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
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
- what IS MDS?
what's another common name for it?
- Myelodysplastic Syndrome
- 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
6. Abnormal chromosomal karytp
7. N/incr cellularity
- How fast does MDS progress?
- not very; insidiously
- What are 2 results of the neutro/thrombocytopenias?
- -Infection prone
- 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?
- DECREASES IN:
- What morphologic changes are seen in platelets in MDS?
- -Giant platelets
-Hyper OR hypolobed
- what's the NUMBERS GAME?
- Used to classify leukemias:
-WHAT %AGE OF ANC are nRBCs?
-WHAT %AGE OF ANC are BLASTS?
(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
4. Biologic response modifiers
5. Bone marrow transplant (not very optimistic tho)
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