Glossary of 3. Chronic Myeloproliferative Disorders

Start Studying! Add Cards ↓

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
Chronic: years
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
3. Panhyperplasia
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
2. Leukoerythroblastosis
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
i.e. t(9,22)
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
-Increased WBCs
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.
-Dysplasia/megakaryocyte fragments.
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?
2-Ionizing radiation
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!

Add Cards

You must Login or Register to add cards