2. Myelodysplastic Syndromes
Terms
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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?
- CFUs
- What does the CFU-GM diff into?
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CFU-Myeloblast or
CFU-Monoblast - What 3 things does the Lymphoid Stem cell diff into?
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Pre-T cell
Pre-B cell
maybe an NK cell - What are the 5 "series" that categorize cells in the BM morphologically?
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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?
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1. Interleukin 1, 3, and 6
2. GM-CSF - What are the 3 Lineage-restricted GFs?
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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?
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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?
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-Infection prone
-Bleeding tendencies - What morphologic changes in RBCs are seen in MDS?
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-Megaloblastoid
-Inclusions
-Dimorphic
-Sideroblastic
-Multinucleation - What metabolic changes in RBCs are seen in MDS?
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-changes in Ag and enzymes
-Acquired Hb H
-Increased Hb F - What morphologic changes indicate dysgranulopoeisis?
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-Pelgeroid, but only 30%
-Mixed eos/basos granules
-Hypogranularity
-Monocytosis - what metabolic chanes occur in the granulocytes?
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DECREASES IN:
-Myeloperoxidase
-LAP
-Chemotactic response
-Bacteriocidal activity - What morphologic changes are seen in platelets in MDS?
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-Giant platelets
-Abnormal granulation
-Circltng micromegakaryocytes
-Hyper OR hypolobed - what's the NUMBERS GAME?
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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?
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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?
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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?
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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?
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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?
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>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?
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-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?
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1. Support - blood transf, platelets, prophylactic antibiot
2. Vitamins/hormones
3. Chemo
4. Biologic response modifiers
5. Bone marrow transplant (not very optimistic tho)