Glossary of 3. Chronic Myeloproliferative Disorders
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- What is a Myeloproliferative Disorder?
- A clonal neoplastic disease of pluripotent hematopoietic stem cells.
- How many acute myeloprolif disorders are there, and what are they?
1. AML (myeloblastic)
2. APL (progranulocytic)
3. AML (myelomonocytic
4. AML (monocytic)
5. AEL (erythroleukemia)
6. AML (megakaryoblastic)
- How many chronic myeloprolif disorders are there, what are they?
1. CML/CGL (myelogenous and granulocytic)
2. PV (Polycythemia Vera)
3. Essential Thrombocythemia (ET)
4. Idiopathic Myelofibrosis (IMF)
- How soon do you die in Acute vs. Chronic leukemia?
- Acute: months
- What cell types characterize ACute vs. Chronic?
- Acute = blasts
Chronic = mature
- What type of organomegaly is seen in Acute vs. Chronic?
- Acute = mild
Chronic = severe
- How does platelet count vary between Acute vs. Chronic?
- Acute = low platelets
Chronic = elevated-normal
- What are 4 points you have to generally consider/recognize when analyzing myeloprolif leukemias?
- 1. Clonality
2. Transformation btwn disease states
3. Terminal AL
4. Overproduction of any/all cellular components - normal maturation, limited function.
- What proves that AML and CML are clonal disorders? (2 things)
- 1. G6PD expression in females
2. Philadelphia chromosome - the same disorder occurs in all the cells.
- What are 8 features that are common to all the CHRONIC myeloprolif disorders?
- 1. Splenomegaly
2. Marrow fibrosis
4. Disorder transitions
5. Terminal AL
6. Megakaryocytic hyperplasia
7. Cytogenetic abnormalities
8. Insidious onset in people >40yr old
- What are the 7 lab findings in Chronic myelofibrosis leukemias?
- 1. N/N Anemia
3. Hi early/Lo later Plts
4. BM shows panhyperplasia early, fibrosis/osteosclerosis later.
5. Chromosome studies
6. Abnormal LAP levels
- What is LAP?
What is it for?
- Leukocyte Alkaline Phosphatase aka NAP (neutrophil).
Measuring it differentiates CML from leukemoid reactions.
- How does the LAP test work?
- LAP enzymatically hydrolyzes Naphthol, the product takes up dye and color = enzyme activity.
- What's a typical diff like for chronic phase CML?
- WBCcnt = 50,000 - 20,0000; very elevated!!
Few (<5%) Blasts/promyelocytes
Gobs of PMNs/bands/metas
Increased Platelets, Retics, Uric acid/LDH, ME ratio -> there is high turnover of cells.
- What is the LAP level like in CML?
- decreased - there are lots of WBCS, but they aren't activated.
- what is the cause of CML?
- the Philadelphia chromosome abnormality.
- What type of chromosomal abnormality is the Ph'?
- A translocation between 9 and 22
- is there a Ph' abnormality in juvenile CMH?
- no - it's a very aggressive disease.
- How is CML differentiated from leukemoid and other CMPDs?
- by the low LAP.
- What are 3 lab findings in accelerated CML?
- -Increased immature cells
-Increased basophilia - mkd
- What changes take place in the lab findings in Acute/Blast crisis CML?
- -Platelets decrease (instead of increased like earlier)
-Additional chromosome abnorml.
-SEVERE anemia, basophilia
-Blasts INCREASE in PB/BM, etc
-Pancytopenia b/c of marrow fibrosis.
- What blast types increase in AML acute phase?
- it's VARIABLE - which ones increase determines the prognosis.
- What type of blast increase has a better prognosis in patients with CML?
- If the lymphoid line increases. 20-30% Lymphoid blasts can be treated to go into remission.
- What blast lineage has worse prognosis for CML during acute phase?
- Myeloid blasts - this increases in the acute phase in 60-65% of cases, and is untreatable.
- Other than Lymph/Myeloid blast increases, what 3rd type of blast increase can occur?
- Bi-phenotypic - mixed lineage or undifferentiated blasts.
- what are 5 possible causes of CML?
- 1-Ph' translocation, viral?
3-Alkylating agents (chemo)
4-Biologically active chemicals
- what are 2 general categories of symptoms in CML, what are they?
- 1. Anemic - SOB, malaise, fatigue, flu-like.
2. Marrow expansion/organomegaly - bones ache, full belly, retinal hemorrhage, hematuria/purpura.
- What is the incidence of CML?
- -20% of ALL the leukemias,
Kills 1:100,000 ppl
-More men than women slightly
-mostly 30-50 yr olds.
- what clinical symptoms are seen in CML based on phase?
- Chronic = asymptomatic, 6 yrs
Acceltd = symptoms, 1-4 yrs
acute/blast = few wks-months
- what are some ways to treat CML?
- -Conventional - chemo
-Aggressive: bm transplant, IFN gamma therapy
-New: Bcr0abl tyrosine kinase inhibitors
- What causes the splenomegaly in chronic leukemia?
- extramedullary hematopoiesis
- What does chronic leukemia often terminate in?
- Acute leukemia
- What kind of blasts are present in accelerated/blast transformed CML?
- Usually myeloid, but could be lymphoid -> it's a stem cell abnormality!
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