Hem Test 2: Leukemia, Bench To Bedside

Front 1

Neoplasms of hematopoeitic cells

Back 1

leukemia

Front 2

2

Back 2

common lymphoid progenitor
common myeloid progenitor

Front 3

ALL origin

Back 3

committed lymphoid progenitor cell (pre-T or pre-B)

Front 4

CLL origin

Back 4

malignancy of mature B cells

Front 5

AML origin

Back 5

malignancy of committed myeloid progenitor cells

Front 6

which leukemia is a myeloproliferative disorder?

Back 6

CML (like p vera, ET, MF)

Front 7

What do the malignant cells maintain in CML?

Back 7

ability to differentiate.

Front 8

Risk factor/incidence for CML?

Back 8

median age of 66yo
male slightly more
risk factor of ionizing radiation exposure

Front 9

Common lab findings of CML

Back 9

leukocytosis, anemia, thrombocytosis

Front 10

Common symptoms of CML

Back 10

HSM, fatigue, weight loss, abdominal fullness

Front 11

Differentiation between CML and p.vera

Back 11

anemia in CML v. erythrocytosis

Front 12

what is present in 95% of CML?

Back 12

Philadelphia chromosome
t(9:22)
(required for dx)

Front 13

Product of Philadelphia chromosome

Back 13

bcr-abl

Front 14

Dx approach to CML

Back 14

peripheral smear and bone marrow aspirate

Front 15

Best screening test for CML

Back 15

FISH
(neg is doesn't look for other abnormalities)

Front 16

Best for picking up bcr-abl

Back 16

quantitative PCR testing

Front 17

3 phases of CML

Back 17

chronic and advanced (accelerated (6-9m), then blast crisis(3-6m))

Front 18

Therapeutic options for CML

Back 18

Abl tyrosine kinase inhibitors
-Imatinib (Gleevec)
-2nd generation Abl inhibitors (Nilotinib, Dasatinib)

Front 19

Mechanism of Gleevec

Back 19

binds to inactive conformation of BCR-ABL

Front 20

Which resistance is more common for Imatinib?

Back 20

Secondary (most likely is presence of point mutations within ABL kinase domain, 90%)

Front 21

When in Nilotinib used?

Back 21

after point mutations on Gleevec

Front 22

Graft v. host in CML

Back 22

associated with decrease risk of relapse

Front 23

Demo of CLL

Back 23

median age 72
risk factors: familial, Agent Orange

Front 24

Lab findings of CLL

Back 24

leukocytosis (lymphocytosis)
anemia
thrombocytopenia
hypogammaglobulinemia

Front 25

Common symptoms of CLL

Back 25

HSM, lymphadenopathy, frequent infections (respiratory and/or encapsulated organisms)

Front 26

What can present in CLL?

Back 26

autoimmune hemolytic anemia (10-25%)
Immune thrombocytopenia (2%)

Front 27

CLL can transform to what two neoplasms?

Back 27

Richter's transformation (large cell lymphoma)
Prolymphocytic leukemia (10% of CLL)

Front 28

What is overexpressed in CLL?

Back 28

Bcl-2 (inhibitor of apoptosis, inhibits release of cytochrome c)

Front 29

Indications for chemo in CLL

Back 29

progressive symptoms related to disease
progressive adenopathy or organomegaly
autoimmune cytopenias
inc frequency of bacterial infections

Front 30

4 classes of chemo for CLL

Back 30

alkylating agents
corticosteroids
purine analogs
monoclonal antibodies

Front 31

AML demographics

Back 31

more deadly
median age: 68
Risk factors: prior radiation, prior chemo (alkylating or Topoisomerase II inhibitors), benzene exposure
prior hx of MDS, familial syndromes

Front 32

compare presentation of AML to CML and CLL

Back 32

much more rapid onset of symptoms

Front 33

clinical manifestations of AML

Back 33

hypermetabolic syndromes
anemia
neutropenia
thrombocytopenia
hyperleukocytosis
HSM (not as much lymphadenopathy)

Front 34

Acute presentations of AML

Back 34

Acute DIC, extramedullary involvements (leukemia cutis)

Front 35

Class I mutations in AML lead to what?

Back 35

proliferation and survival

Front 36

Class II mutations lead to what?

Back 36

impaired differentiation

Front 37

Type I mutation in AML

Back 37

FLT-3 activating mutations

Front 38

Type II mutation?

Back 38

retinoic acid receptor-alpha, form fusion gene PML-RAR alpha (can tx with retinoids)

Front 39

Prognosis for AML

Back 39

-Bad if older
-bad if complex, deletions of chromosomes 5&7, 3q abnormalities
-bad if arising from prior hx of MDS
-bad if tx related AML (prior radiation)

Front 40

Tx for APL (variant of AML)

Back 40

induction chemo + all-trans retinoic acid (differentiating agent)

Front 41

Most common pediatric cancer

Back 41

acute lymphoblastic leukemia

Front 42

demo of ALL

Back 42

median age: 11
risk factors include prior radiation, prior chemo, familial syndromes

Front 43

What can cause acute renal failure in ALL?

Back 43

uric acid deposition in kidneys from high cell turnover

Front 44

Mediastinal mass indicates what?

Back 44

precursor T-cell ALL

Front 45

Type I mutations of ALL

Back 45

t(9:22), the Philadelphia chromosome

Bcr-Abl associated with worse prognosis (more common in adult cases)

Front 46

Type II mutations in ALL

Back 46

t(12,21)
more common in childhood B-cell ALL

associated with good prognosis

Front 47

Bad indicators of prognosis of ALL

Back 47

<1,>10yo
t(9:22), 11q23 (MLL) translocations
hypodiploidy
high WBC count

Front 48

ALL stages of tx

Back 48

induction
consolidation
interim maintenance
delayed intensification
maintenance

Front 49

Severe cytopenias often indication what?

Back 49

acute leukemias

Front 50

Seeing blasts on smear indicates what?

Back 50

AML or ALL

Front 51

Seeing mature lymphs on smear indicates what?

Back 51

CLL

Front 52

When is DIC more common?

Back 52

AML

Front 53

When seeing the spectrum of myelopoiesis, what does that indicate?

Back 53

CML