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49 Cards in this Set

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What is the exact opposite of anemia?
Erythrocytosis
What is the definition of Erythrocytosis? Another quasi-name?

What are the two classifications of causes?
An increase in the number of circulating RBCs per volume of blood.
Synonym = polycythemia

Primary, Secondary
What causes Secondary Erythrocytosis?
tissue hypoxia --> EPO production by the kidney
Can treatment with EPO and/or androgens cause pirmary or secondary erythrocytosis?
secondary
What is hyperviscosity syndrome? This is developed by some patients with...
Headaches
Visual changes
Tinnitus
Dizziness
Paresthesias
Decreased mental acuity

...erythrocytosis.
What does the decision to treat secondary erythrocytosis depend on?
prescence of hyperviscosity syndrome. yes = treat, no = don't treat.
Define/Characterize the myeloproliferative disorders. Are they clonal or non clonal?
Stem Cell Disorders leading to autonomous production of hematopoietic cells from ALL THREE LINEAGES (red cells, white cells, platelets)?

Clonal.
Name the neoplastic disorder arising from a pluripotent stem cell, generally characterized by erythrocytosis, with or without concomitant thrombocytosis and leukocytosis.

Is this an example of primary or secondary erythrocytosis?
Polycythemia vera.

primary.
Primary vs. Secondary Erythrocytosis?
Primary: BM is doin' whatever it wants.

Secondary: BM is doing what it's told.
In P. vera, many if not all of the circulating blood cells are derived from a ______ neoplastic stem cell? Why is this?
single

They can divide w/o EPO.
How do we Dx. P. vera these days?
We do two tests:
EPO = it will be low.

JAK2 mutation
Will EPO be low or high in p. vera?
low.
JAK2 V617F causes loss of _______ leading to _____ ____ of JAK 2.

Is this seen in normal people?
auto-inhibition

constitutive activation

Never.
What the 4 phases of the progression of P vera?
Latent phase - asymptomatic

Proliferative phase - pts may be hypermetabolic or have sx of hyperviscosity or thrombosis

Spent phase - anemia, leukopenia, secondary myelofibrosis, increasing liver and spleen size

Secondary AML
1-2% of pts treated with phlebotomy alone
Certain drug therapies increase risk
What is the 'spent' phase of P vera?
patients have anemia, thrombocytopenia
liver enlarged, splenomegaly.

Marrow is filled with scar tisue, liver and spleen start showing hematopoesis.
What are 6 Sx more specific to P vera and myeloproliferative diseases?

* note that all of the other erythrocytosis sympt still apply in addition.
Pruritis after bathing
Erythromelalgia
Hypermetabolic symptoms
Thrombosis (arterial or venous)
Hemorrhage
What is Erythromelalgia?
platets produced are too active
PEx findings in P vera?

Lab values?
Facial Plethora
Splenomegaly
Hepatomegaly
Distention of Retinal veins.

elevated platelets,
basophilia (can be seen in any MPD)
LOW EPO lvls
+ JAK2 V617F

elevated uric acid, elevated B12
elevated leukocyte alkaline phosphatase
P vera Tx?

What has shown to decrease risk of thrombosis in PV patients?
Phlebotomy until they're iron deficient.

Hydroxyurea

Low dose aspirin, give it to all patients.
What are the downsides of treating P vera w/ phlebotomy?
Increased risk of thrombosis

No effect on progression to spent phase

May be insufficient to control disease
What is the wimpier sibiling of P.vera?

What % is clonal?

Those that aren't clonal, explain?
ET: essential thrombocythemia

50%

the rest have polyclonal increase in megakaryocytes
How do we make the Dx of essential thrombocythemia (ET)?
First, rule out secondary causes of thrombocytosis: cancer, infection, inflammation, bleeding, iron deficiency

Exclude P vera
Exclude CML

Platelets must be >600 on two separate occasions
Does ET progress to AML? What is the major complication of ET?

Can ET progress to myelofibrosis?
No, rarely.

thrombosis.

Yes, it can.
Thrombosis in ET is mainly which type?
You see both arterial and venous.
Labs in essential thrombocythemia (ET)?

Which percentage have a JAK2 mutation?
If the plt count is very high, there may be pseudohyperkalemia and pseudohypoglycemia. This goes away if the blood is drawn into a heparinized tube.

50%.
What is the Dx if patients have a + JAK 2 and high platelets?
ET is the Dx
What are pseudohyperkalemia and pseudohypoglycemia? when is this seen?
platetes eat the sugar in the tubes where there is no heparin... can make the potassium & glucose artifically high.
What is this?
A giant platelet.
What are these? When are they seen?
abnormal megakaryocytes, ET.
ET Tx? Who do we Tx?

How do we tx?
Treatment targeted at reducing the platelet count

those who have had or are at risk for thrombosis, those >65 y.o., or pts with plts > 1-1.5 million

Anagrelide
Hydroxyurea
Interferon alpha
In Myelofibrosis (MF), why can't the patients eat even though they're hungry?
Spleen is HUuuuuGE, 'n takes up the space.

Hepatomegaly too.
MF peripheral smear shows....
leukoerythroblastic picture, with teardrops, NRBC and early granulocytes
"Dry tap" is what? associated with what?

What is seen on BM biopsy?
inability to aspirate liquid marrow

MF

Increased collagen and reticuluin
What is the Tx of MF?

Is splenectomy a good idea?
There is no definitive therapy

If patient is young, BM transplant can be done, but older patients have too high mortality

well, it's very risky., frequent complications of thrombosis, hemorrhage, and infection.
What does 'myelo-' mean?
marrow
Define myelodysplastic syndromes.
Conditions in which there is disordered maturation in 1 or more cell lines, usually producing cytopenias
Population of dysplastic cells represent an abnormal clone of cells
Myelodysplastic syndromes are a ____ of disorders. Are they inherently progressive?
spectrum

No.
Are myelodysplastic syndromes (MDS) a disease of the old or the young?
elderly.
What are the lab findings in MDS?
Macrocytosis w/o low B12 or folate

neutrophils can be hypogranular or bi-lobed

can have monocytosis (don't know why)
How should we think about MDS?
zerox, or a zerox, of a zerox.... and eventually (when you're old) you can't tell a c from an a, etc.

In the case of MDS, we have cells, but they're not quite right.
What are some features of the marrow in MDS?
Megaloblastic erythropoeisis

Ringed sideroblasts

Abnormal nucleus of RBC precursors (dyserythropoiesis)
Small megakaryocytes with abnormally hypolobate nuclei
Blast cells should account for <20% of marrow cells
In which disorder should blast cells account for <20%?

>20%?
MDS.

Acute leukemia.
Where are ringed sideroblast seen?
in MDS.
___ - - - progression ---- -- -- > Acute Leukemia?
MDS
Tx of MDS?

Once the patient develops AML, the chacnes of achieving remission is _____, and their duration of any remission is ______.
usually supportive: transfustions of RBCs and platelets, Growth Factors

decreased, shorter.
If you're gonna get AML, whould you rather get it as a young person de novo, or as an old person as the end of a progression of MDS? Why?
young person, because they kid's other stem cells are probably ok. those old people with progressive MDS probably have corrupted their other cells as well.
What is a clonal stem cell disorder that affects primarily megakaryocytes?
Myelofibrosis (MF)
The spent phase of all myeloproliferative disorders can be difficult to distinguish from...
...primary MF
What type of cell is shown here? What is it characteristic of and why?
Ringed sideroblast. Myelodysplastic Syndrome (MDS).
The body has iron available but cannot incorporate it into hemoglobin.