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Hematology > Myeloproliferative Diseases > Flashcards

Myeloproliferative Diseases Flashcards

1
Q

What are the four myeloproliferative syndromes?

A

Chronic Granulocytic Leukemia
Idiopathic Myelofibrosis
Primary Thrombocytopenia
Polycythemia Ruba Vera

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2
Q

Explain the epidemiology of CML

A

Median age = 45-55
Incidence increases with age 12-30% above 60
Slightly more men than women
At presentation: 85% ddx in chronic phase

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3
Q

What are the three etiologic factors associated with Chronic Granulocytic Leukemia?

A

Idiopathic
Radiation
Chemical

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4
Q

What are symtoms of Chronic Granulocytic Leukemia?

A 
Fatigue
Weight Loss
Abdominal Fullness
Easy bruising or bleeding
Abdominal Pain
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5
Q

What are physical findings of CGL?

A 
Splenomegaly
Hepatomegaly
Sternal Tenderness 
Purpura
Retinal Hemmorhage
Fever
Palpable Lymph Nodes
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6
Q

What are some common lab findings in CML?

A 
Elevated Uric ACid
Low Leukocyte Alkaline Phosphatase Score
Elevated b12
b12 binding protein elevation
transcobalamine 12
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7
Q

What are the three phases of CML?

A

Chronic Phase (median 5-6 yrs)
Accelearated Phases 6-9 mnts
Blast crisis median survival 3-6 months

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8
Q

What is the classic molecular abnormality in chronic myelogenous leukemia?

A

Philadelphia Chromosome

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9
Q

What is the philadelphia chromosome?

A

9/22 translocation
found in 95% of patients with CML
Present in 100% of RBC WBC MONOCYTES AND PLTS

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10
Q

What does the philadelphia chromosome do?

A

It makes high levels of tyrosine kinase activity at bcr-abl

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11
Q

What did preclinical studies about the philadelphia chromosome determine?

A

By putting bcr-abl in mice, they were able to produce the disease

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12
Q

What are drugs that control cml?

A

hydroxyurea, interferon and targetted agents, imatinib

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13
Q

What is the significance of allogeneic sct in cml?

A

good match produces 60% survival, only tx available, transplants have become less common

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14
Q

How does imatinib work?

A

It blocks the atp receptor at the bcr-abl tyrosine kinase, preventing abnormal proliferation

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15
Q

What are the characteristics of imatinib?

A

rapidly absorbed after oral admin
absolute bioavailability at 98%
terminal half-life = 18-22 hrs

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16
Q

What were the results of imatinib phase one trials/

A

They worked in ifn-a resistant and blast phase patients. in a month peripheral blood returned to normal

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17
Q

Whatdoes taking imatinib do if you take it longer?

A

The longer you use it, the longer you stay in remission.

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18
Q

what do you treat with in a px with resistnace in imatinib?

A

BM transplant
Dasatinib
Nilotinib

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19
Q

What are the consequences of using dasatinib and nilotinib over imatinib/

A

they get to remission faster but less studied, both are approved for first line use.

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20
Q

What are the jak2 inhibitors

A

prv, mf, and tt

21
Q

Explain the epo, epor, and jak2 relationship

A

epo and epor are crucial in rbc formatino
jak2 is bound to epor and activated upon epo binding to the receptor
signaling of epor starts with jake2activation.
epor signaling activates stat, mapk, pi3kinase and akt
results in proliferation and differentiation of rbc precursors

22
Q

What are the three common mutations associated with jak2?

A

pv, et, and mf in order of decreasing frequency in cml

23
Q

What is the general characteristic of primary myelofibrosis?

A

bm has fibrous tissue so bm made in liver and spleen

24
Q

what is the median age of pmf onset?

A

65

25
Q

what are the causes of pmf?

A

myeloproflieration with granulocytic hyperplasia and megakaryocytosis
marrow fibrosis with increased collagen 1,3,4,5
dysmorphic megakaryocytes
fibrosis is the result of release of cytokines from the abnormal megakaryocytes

26
Q

What are the clinical symptoms of pmf?

A

fatigue, weakness, dyspnea, palpitations, weight loss, early satiety, pain in left shoulder, bone pain in lower extrem

27
Q

what are the physical findings of pmf?

A 
hepatosplenomegaly
cachexia
peripheral edema
purpura
bone tenderness
neutrophilic dermatosis
28
Q

what is the primary histopath associated with pmf?

A

tear drop cells, aniso and poikliocytosis, nucleated rbc and wbcs, marrow has fibrosis

29
Q

What commonly occurs in many placesi n the body when someone has pmf?

A

fibrohematopoeitic extramedullary tumors

30
Q

what are two big symptoms of pmf?

A

portal htn

and osteosclerosis

31
Q

what are common therapies in pmf?

A

androgens, procrit, hydroxyurea, thalidomind, lenalidamide,radiation, splenectomy, bm transplant

32
Q

What is the least common of the mpds?

A

essential thrombocytosis

33
Q

what is the average age of et?

A

50-60

34
Q

what are the clinical features of thrombocytosis

A

fever, night sweats, weight loss, splenomegally, aortic andmitral valvular leaflet thickening or vegetations, leukemic transformation is less than the other mpds

35
Q

on histo, what do you see in et?

A

giant plts and massive megakaryocytes

36
Q

Tell me about the serum thrombopoeitin in et

A

levels are normal or elevated and doesn’t coorelate with plts count.

37
Q

what happens with thrombosis and bleeding in et?

A

50/50 split between the two. half have non-fxn plts, others have overactive plts.

38
Q

what are the risk factors for thrombosis in et

A

previous thrombosis, advanced age, cv risk factors ie smoking

39
Q

what is the risk factor for bleeding with et?

A

greater than 1500000 plts

40
Q

what are thrombotic complications of et

A

50% of patients have at least one peisode in 9 years of follow up
arterial throm is more common
venous complics usually of lower extrem
arterial sites are cva, coronary arter disease
placental insufficiency
budd-chiari syndrome

41
Q

how do you treat et?

A

hydroxyurea (interferes with s phase progressino of dna synthesis.

anagrelide
interefers with terminal differentiation of megakaryocytes,

42
Q

What are the causes of excess production of epo?

A

cellular hypoxia

local renal hypoxia (ligation, plaques, transplant rejection, pressure of kidney parenchyma)

43
Q

What are characteristics of polcythemia ruba vera

A

more common in men, commonly in 50-60.

44
Q

what are symptoms of polycytehmia vera?

A

headache, weakness, pruritus, dizziness, sweating, weight loss, more.. rosacea, conjunctival suffusion

45
Q

what are the lab findings of prv?

A 
hct 55-65
2/3 have leukocytosis
2/3 have basophilia
lap score and b12 levesl elevated in 70% of pxs
marrow hypercellularity
absence of iron stores
46
Q

what are the thrombotic complications of prv?

A

1/3 have events
budd-chaiari occurs in 10%
erythrombelalgia (warm extremeties, painful digits, burning senstion)

47
Q

What are the therapies for prv?

A

phlebotomy

myelosuppresive agents whe plts and wbc rises

48
Q

what is the spent phase of prv?

A

anemia, leucocytosis marrow fibrosis enalrgin spleen, at this stage mimics mf

Hematology (7 decks)

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