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IHO Week 4 > Chronic myeloproliferative disorders (CML) General Info > Flashcards

Chronic myeloproliferative disorders (CML) General Info Flashcards

1
Q

What cells are proliferating in chronic myeloproliferative disorders?

A

myeloid cells - NOT blasts, but maturing cells

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2
Q

Who do chronic myeloproliferative disorders occur in?

A

only adults

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3
Q

Do the chronic myeloproliferative disorders tend to have a long or short course?

A

long course

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4
Q

What are the four chronic myeloproliferative disorders?

A

chronic myeloid leukemia
polycythemia vera
essential thromobocythemia
myelofibrosis

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5
Q

What cell proliferates the most im CML?

A

neutrophils - with left shift

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6
Q

What cells proliferate the most in polycythemia vera?

A

red cells

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7
Q

What cells proliferate the most in essential thrombocythemia?

A

platelets in blood and megakaryocytes in bone marrow

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8
Q

What cells proliferate the most in myelofibrosis?

A

trick question - they all proliferate. the key symptoms is that the bone marrow becomes fibrosis

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9
Q

What’s the general underlying defect in all the chronic myeloproliferative disorders?

A

something goes wrong in a stem cell early in the pathway and it presents along the myeloid arm - so you get an increased WBC with left shift in all of them

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10
Q

WHat type of protein is mutated in the chronic myeloproliferative disorders?

A

tyrosine kinases

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11
Q

What organ typically is enlarged in the chronic myeloproliferative disorders?

A

the spleen

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12
Q

CML presents with neutrophilia and an increase in what other blood cell?

A

basophilia (it’s baically the only thing that will cause this)

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13
Q

What’s the chromosome in CML/

A

philadelphia chromosome

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14
Q

Will hemoglobin be up or down in CML?

A

down

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15
Q

What will platelet count do in CML?

A

go up initially, but as disease progresses it will decrease

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16
Q

What is LAP? Is it up or down in CML and why?

A

It’s leukocyte alkaline phosphatase - expressed in healthy neutorphils, but not in malignant neutrophils

So you get a decrease in LAP in CML - it’s a way to differentiate the neutrophilia from CML and just a really bad infection with left shift

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17
Q

What’s the best test for CML?

A

PCR for the philadelphia chromosome

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18
Q

What are the symptoms of CML?

A

slow onset (often asymptomatic) fever, fatigue, night sweats, abdominal fullness

19
Q

What are the three phases of CML?

A
  1. chronic phase
  2. accelerated phase
  3. blast crisis
20
Q

What are the characteristics of the chromic phase?

A

stable blood counts
easily controllable
usually 3-4 years if untreated

21
Q

What happens in the accelerated phase?

A

counts become rapidly unstable and you’re in blast crisis within 6-12 months

22
Q

What is blast crisis?

A

basically it switched to acute leukemia - super high mortality

23
Q

Why can people with CML in the blast phase develop and acute LYMPHOCYTIC leukemia?

A

because remember the defect is in a stem cell - the disease just seems to present in the myeloid arm until this stage

24
Q

What are the 3 levels of remission in CML?

A 
hematologic remission (WBC less than 10,000, normal morphology, normal Hb, normal platelets, no splenomegaly)
Cytogenetic remission (no metaphases with T9:22)
Molecular remission (no BCR/ABL by PCR - this is the one you want)
25
Q

What are the major emergencies that can occur with polycythemia vera?

A

thrombosis and hemorrhage

26
Q

What’s mutated in almost all cases of PV?

A

Jak-2

27
Q

WHat’s the difference between a primary and secondary polycthemia vera?

A

primary - an intrinsic myeloid cell problem

secondary - due to increased erythropoietin due to some sort of O2 lack

28
Q

WHat are the diagnostic criteria for A (maor) PV?

A

increased red cell mass
normal O2 saturation
splenomegaly

29
Q

What are the B (minor) criteria for PV?

A
  1. thrombocyosis
  2. increased WBC without infection
  3. increased LAP (unlike CML)
  4. Increased B12 (we don’t know why)
30
Q

What are the clinical symptoms and signs for PV?

A

headache, pruritis, dizziness, thrombosis, infarction, splenomegaly, hepatomegaly, plethora (the blood pools in the face and you look red)

31
Q

Is Jak-2 activity increased or decreased in PV?

A

increased - so the RBCs can proliferate on their own

32
Q

What are the main treatment goals for PV?

A

phlebotomy - you gotta get the bloo dout because it’s so sludgy they’ll die form thrombosis

maybe myelosuppressive drugs

33
Q

What’s the prognosis for PV?

A

9-14 years, death from thrombosis or hemorrhage

34
Q

What’s high in essential thrombocythemia?

A

platelets in the blood, megakaryocytes in the bone marrow

35
Q

Who gets essential thrombocythemia?

A

yunng women can get it

36
Q

What are the diagnostic criteria for thrombocythemia (remember it’s a diagnosis of exclusion)?

A 
plateelet count over 600,000
Hb less than 13 or RBC mass normal
no philadelphia chromosome
no marrow fibrosis
no othe rreason for thrombocytosis (because this is a super rare disorder)
37
Q

What are the clinical symptoms and signs of essential thrombocythemia?

A

bleeding (because the platelets don’t work properly), thrombosis, purpura, bruising, pallor, tacchycardia, and enlarged spleen

38
Q

What’s the treatment for essential thrombocythemia?

A

platelet pheresis and maybe myelosuppressive drugs

aspirin to avoid clotting

39
Q

What’s the median survival of ET and what’s the usual cause of death?

A

5-8 years - thrombosis or hemorrhage

40
Q

What’s the main clinical feature of chronic myelofibrosis?

A

Panmyelosis (everything proliferates) and then the marrow becomes fibrotic

41
Q

What are the clinical signs and symptoms of chronic myelofibriosis?

A

left upper quadrant fullness

weakness, fatigue, palpitations, huge spleen (biggest of all these disorders), pallor, tachycardia

42
Q

What odd shape of RBCs can be seen in chronic myelofibrosis?

A

teardrop RBCs - because they have to pull their way out of the fibrotic bone marrow

43
Q

What’s the teratment for chronic myelofibrosis?

A

supportive basically- maybe myelosuppressive drugs early on

44
Q

What’s the prognosis in MF? cause of death?

A

3-5 years from marrow failure

IHO Week 4 (15 decks)

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