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09 Hematology > Malignancies 1 > Flashcards

Malignancies 1 Flashcards

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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
characteristics of essential thrombocythemia
- clonal disorder involving hematopoietic progenitor cells - accumulation of phenotypically normal platelets - mutations in jak2 v617f, mpl, and calr
26
causes of thrombocytosis
``` tissue inflammation (collagen vascular disease, ibd) malignancy infection myeloproliferative disorders myelodysplastic disorders postsplenectomy or hyposplenism hemorrhage iron deficiency anemmia surgery rebound hemolysis familial ```
27
diagnostic criteria for essential thrombocythemia
7 items, table 5
28
effects of essential thrombocythemia
high platelets --> at risk for hemorrhagic episode because of consumption of all the von willebrand factor
29
treatment for essential thrombocythemia
platelet pheresis | hydration
30
treatment for hemorrhagic episodes in essential thrombocythemia
``` hydroxyurea* anagrelide to inhibit megakaryocyte differentiation* interferon alpha alkylating agents antiplatelet agents* ```
31
essential thrombocythemia can lead to ___ and ___
myelofibrosis and aml
32
essential thrombocythemia low risk
age <60 and no prior cv event m: nil or low dose aspirin
33
essential thrombocythemia intermediate risk
generic cv risk factors m: low dose aspirin
34
essential thrombocythemia high risk
age >60 with prior cv event m: myelosuppression + low-dose aspirin
35
characteristics of myelodysplastic syndrome
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
dysplastic features in myelodysplastic syndrome
binucleated rbcs myeloblast in peripheral smear hypersegmentation of neutrophils
37
treatment for myelodysplastic syndrome
``` transfusion, chelation hematopoietic growth factor demethylating agents lenalidomide immunosuppression chemo allogeneic stem cell transplantation!! ```
38
t/f younger people are excluded from getting aml
false
39
risk factors for aml
hereditary, radiation, drug, chemical or other occupation exposure, idiopathic
40
features of aml
``` anemia: easy fatigability leukopenia/leukocytosis leukocyte dysfunction: fever, susceptibility to infections thrombocytopenia: bleeding, hemorrhages organomegaly ```
41
peripheral smears of aml
very large myeloblasts
42
peripheral smear of acute promyelocytic leukemia
stick like figure called rods in faggot cells | butt cells
43
better risk results in cytogenic testing of aml
inv 16, t(16,16), t(8,21), t(15,17) very responsive to treatment, 55-65%
44
intermediate risk in cytogenic testing in aml
normal cytogenetics, t(9,11) 24-40%
45
poor risk in cytogenic testing in aml
complex karyotypes, deletion of 5, 7, 5q, 7q very aggressive treatment, 5-14%
46
most important diagnostic test for aml
cytogenic testing
47
other diagnostics for aml
``` pretreatment evaluation morphology (smear) cytochemical analysis (myeloperoxidase, nonspecific esterase) immunophenotyping flow cytometry cytogenetics ```
48
principle of flow cytometry
determines the cell signature of the leukemia for classification via CD antigens dictates prognosis and treatment
49
definitive antigens used for aml
cytoplasmic mpo
50
antigens used for all b-cell
cytoplasmic cd 79a, cd22, cd19, cd10
51
definitive antigens used for all t-cell
cytoplasmic cd3 surface cd3 t cell receptor
52
treatment for aml
first determine fitness (young, good cv = chemo)
53
phases of chemo for aml
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
phases of chemo in apl
induction - all trans retinoic acids, arsenic trioxide, anthracycline consolidation phase - atra, arsenic trioxide, anthracycline maintenance phase -tablets, supportive therapy
55
risk factor for chronic myeloid leukemia
radiation 15% of all leukemias
56
patho in chronic myeloid leukemia
formation of philadelphia chromosome = bcr-abl gene combination --> new tyrosine kinase
57
downstream signaling pathways in cml
table 10
58
features of cml
``` asymptomatic anemia splenomegaly thrombotic or vaso-occlusive events due to high wbcs blastic accelerated phase ```
59
chronic phase of cml
<10% of the cells in the blood and bone marrow are blast cells (immature)
60
accelerated phase of cml
table 11
61
blast crisis/ blast accelerated phase of cml
blasts are >20% of peripheral blood wbc or nucleated bone, like acute leukemia
62
peripheral blood smears in cml
numerous wbc apparent | wbcs are mature (aml = immature)
63
treatment options for cml
``` leukapheresis/hydroxyurea (not definitive treatments) tki interferon alpha (pregnant) chemo (blastic phase) bone marrow transplant (blastic crisis) ```
64
indication for imatinib
all phases
65
indication and toxicity of dasatinib
i: all phases t: pleural and pericardial effusions, pulmo hpn
66
indication of nilotinib
all except blastic t: vasoocclusive disease, pancreatitis
67
indication for bosutinib and ponatinib
all except frontline ponatinib: skin rashes
68
indication for omacetaxine mepesuccinate
failure of >2 tki
69
(cml) partial cytogenetic response is expected by ___
3rd month <35% cells should be neg for philadelphia chromosome
70
(cml) expectation by 6th month
complete cytogenetic response or complete absence of philadelphia chromosome
71
(cml) expectation by 12th month
major molecular response, pcr neg for leukemic products molecularly undetectable leukemia

09 Hematology (9 decks)

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