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09 Hematology > Malignancies 1 > Flashcards

Malignancies 1 Flashcards

1
Q

patho in myeloproliferative neoplasms

A

overproduction of formed and matured blood elements (not precursors) without significant dysplasia

(looks normal but will continue proliferating)

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2
Q

when blood is being produced elsewhere not just in the bone marrow

A

extramedullary hematopoiesis

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3
Q

patients with mpns also have ___ and are at risk for ___

A

have myelofibrosis, at risk for acute leukemia transformation

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4
Q

characteristics of polycythemia vera

A

clonal disorder involving multipotent hematopoietic progenitor cells

accumulation of phenotypically normal rbc, granulocytes and platelets

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5
Q

pathophysio in pv

A

jak2 mutation that makes activates continuous proliferation

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6
Q

s/sx of pv

A 
incidental finding
high hemoglobin or hematocrit
aquagenic pruritus
hyperviscosity
splenomegaly
constitutional symptoms
erythromelagia
thrombotic episodes
hemorrhagic episodes
low serum epo
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7
Q

diagnostic criteria for pv

A

hg, hc or increased red cell mass

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8
Q

relative erythrocytosis

A

hemoconcentration secondary to dehydration, diuretics, ethanol abuse, androgens, or tobacco use

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9
Q

absolute erythrocytosis

A

hypoxia, tumors, renal disease, drugs, familial, pv

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10
Q

what happens in renal artery stenosis

A

blood is not gonig in kidney -> organ feels that it’s not getting enough o2 -> production of epo and blood

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11
Q

patho in hypernephroma or pheochromocytoma

A

tumors can produce catecholamines -> stress -> more rbc production

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12
Q

complications of pv

A

hyperuricemia
thrombotic complications
progression to myelofibrosis (spent phase)
progression to aml

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13
Q

treatment goals for pv

A

men: hgb = 14 g/dl or hct <45%

women hgb = 12 g/dl or hct <42%

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14
Q

treatment for pv

A 
phlebotomy
hydroxyurea (ribonucleotide reductase inhibitor)
interferon alpha
chemo with alkylating agents
radioactive phosphorus (32p)
aspirin/clopidogrel
ruxolitinib
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15
Q

least common myeloproliferative neoplasm

A

primary myelofibrosis

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16
Q

characteristics of primary myelofibrosis

A

jak2, mpl, calr
reticulin fibers in bone marrow
extramedullary hematopoiesis (spleen –> splenomegaly)

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17
Q

constitutinal symptoms of primary myelofibrosis

A

loss of appetite due to compressed stomach caused by splenomegaly
nausea
pruritus, bone pain, night sweat

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18
Q

peripheral blood smear in primary myelofibrosis

A

leukoerythroblastic picture
tear drop cells
nucleated red cells
immature wbcs

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19
Q

bone marrow with silver stain in primary myelofibrosis

A

presence of reticulin fibers

occupy space for hematopoiesis –> pancytopenia

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20
Q

disorders causing myelofibrosis

A

malignant and nonmalignant, table 3

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21
Q

how to diagnose primary myelofibrosis

A

all 3 major criteria and 2 minor criteria, table 4

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22
Q

symptoms of primary myelofibrosis

A

decreased quality of life
increasing marrow failure leading to transfusion dependent anemia
progression to acute myeloid leukemia

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23
Q

therapy for primary myelofibrosis

A 
corticosteroids for low platelets
danazol for hgb
interferon alpha
ruxolitinib to regress spleen and reduce cytokines
allogeneic bone marrow transplant**
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24
Q

disease that results from clonal mutations that lead to the overproduction of mature platelets

A

essential thrombocythemia

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25
Q

characteristics of essential thrombocythemia

A
  • clonal disorder involving hematopoietic progenitor cells
  • accumulation of phenotypically normal platelets
  • mutations in jak2 v617f, mpl, and calr
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26
Q

causes of thrombocytosis

A 
tissue inflammation (collagen vascular disease, ibd)
malignancy
infection
myeloproliferative disorders
myelodysplastic disorders
postsplenectomy or hyposplenism
hemorrhage
iron deficiency anemmia
surgery
rebound
hemolysis
familial
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27
Q

diagnostic criteria for essential thrombocythemia

A

7 items, table 5

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28
Q

effects of essential thrombocythemia

A

high platelets –> at risk for hemorrhagic episode because of consumption of all the von willebrand factor

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29
Q

treatment for essential thrombocythemia

A

platelet pheresis

hydration

30
Q

treatment for hemorrhagic episodes in essential thrombocythemia

A 
hydroxyurea*
anagrelide to inhibit megakaryocyte differentiation*
interferon alpha
alkylating agents
antiplatelet agents*
31
Q

essential thrombocythemia can lead to ___ and ___

A

myelofibrosis and aml

32
Q

essential thrombocythemia low risk

A

age <60 and no prior cv event

m: nil or low dose aspirin

33
Q

essential thrombocythemia intermediate risk

A

generic cv risk factors

m: low dose aspirin

34
Q

essential thrombocythemia high risk

A

age >60 with prior cv event

m: myelosuppression + low-dose aspirin

35
Q

characteristics of myelodysplastic syndrome

A

heterogenous group of myeloid disorders

  • lead to cytopenia
  • then bone marrow failure

can produce blood cells but ugly and ineffective leading to cytopenia

high risk for aml

36
Q

dysplastic features in myelodysplastic syndrome

A

binucleated rbcs
myeloblast in peripheral smear
hypersegmentation of neutrophils

37
Q

treatment for myelodysplastic syndrome

A 
transfusion, chelation
hematopoietic growth factor
demethylating agents
lenalidomide
immunosuppression
chemo
allogeneic stem cell transplantation!!
38
Q

t/f younger people are excluded from getting aml

A

false

39
Q

risk factors for aml

A

hereditary, radiation, drug, chemical or other occupation exposure, idiopathic

40
Q

features of aml

A 
anemia: easy fatigability
leukopenia/leukocytosis
leukocyte dysfunction: fever, susceptibility to infections
thrombocytopenia: bleeding, hemorrhages
organomegaly
41
Q

peripheral smears of aml

A

very large myeloblasts

42
Q

peripheral smear of acute promyelocytic leukemia

A

stick like figure called rods in faggot cells

butt cells

43
Q

better risk results in cytogenic testing of aml

A

inv 16, t(16,16), t(8,21), t(15,17)

very responsive to treatment, 55-65%

44
Q

intermediate risk in cytogenic testing in aml

A

normal cytogenetics, t(9,11)

24-40%

45
Q

poor risk in cytogenic testing in aml

A

complex karyotypes, deletion of 5, 7, 5q, 7q

very aggressive treatment, 5-14%

46
Q

most important diagnostic test for aml

A

cytogenic testing

47
Q

other diagnostics for aml

A 
pretreatment evaluation
morphology (smear)
cytochemical analysis (myeloperoxidase,  nonspecific esterase)
immunophenotyping
flow cytometry
cytogenetics
48
Q

principle of flow cytometry

A

determines the cell signature of the leukemia for classification via CD antigens

dictates prognosis and treatment

49
Q

definitive antigens used for aml

A

cytoplasmic mpo

50
Q

antigens used for all b-cell

A

cytoplasmic cd 79a, cd22, cd19, cd10

51
Q

definitive antigens used for all t-cell

A

cytoplasmic cd3
surface cd3
t cell receptor

52
Q

treatment for aml

A

first determine fitness (young, good cv = chemo)

53
Q

phases of chemo for aml

A

induction phase

  • induce remission, eliminate cancer
  • anthracycline + cytarabine
  • demethylating agents, bcl inhibitors

consolidation phase

  • prolong remission
  • high dose cytarabine or hematopoietic stem cells (allegeneic)
54
Q

phases of chemo in apl

A

induction
- all trans retinoic acids, arsenic trioxide, anthracycline

consolidation phase
- atra, arsenic trioxide, anthracycline

maintenance phase
-tablets, supportive therapy

55
Q

risk factor for chronic myeloid leukemia

A

radiation

15% of all leukemias

56
Q

patho in chronic myeloid leukemia

A

formation of philadelphia chromosome = bcr-abl gene combination

–> new tyrosine kinase

57
Q

downstream signaling pathways in cml

A

table 10

58
Q

features of cml

A 
asymptomatic
anemia
splenomegaly
thrombotic or vaso-occlusive events due to high wbcs
blastic accelerated phase
59
Q

chronic phase of cml

A

<10% of the cells in the blood and bone marrow are blast cells (immature)

60
Q

accelerated phase of cml

A

table 11

61
Q

blast crisis/ blast accelerated phase of cml

A

blasts are >20% of peripheral blood wbc or nucleated bone, like acute leukemia

62
Q

peripheral blood smears in cml

A

numerous wbc apparent

wbcs are mature (aml = immature)

63
Q

treatment options for cml

A 
leukapheresis/hydroxyurea (not definitive treatments)
tki
interferon alpha (pregnant)
chemo (blastic phase)
bone marrow transplant (blastic crisis)
64
Q

indication for imatinib

A

all phases

65
Q

indication and toxicity of dasatinib

A

i: all phases
t: pleural and pericardial effusions, pulmo hpn

66
Q

indication of nilotinib

A

all except blastic

t: vasoocclusive disease, pancreatitis

67
Q

indication for bosutinib and ponatinib

A

all except frontline

ponatinib: skin rashes

68
Q

indication for omacetaxine mepesuccinate

A

failure of >2 tki

69
Q

(cml) partial cytogenetic response is expected by ___

A

3rd month

<35% cells should be neg for philadelphia chromosome

70
Q

(cml) expectation by 6th month

A

complete cytogenetic response or complete absence of philadelphia chromosome

71
Q

(cml) expectation by 12th month

A

major molecular response, pcr neg for leukemic products

molecularly undetectable leukemia

09 Hematology (9 decks)

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