Reading...
Play button
Play button
Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
33 Cards in this Set
- Front
- Back
|
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?
|
SIX:
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?
|
FOUR:
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 5-Idiopathic |
|
|
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 death |
|
|
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!
|
