03.24 - Pathogenesis and Dx (Strom)

Question 1

Alkylating agents predispose to

Answer 1

AML (especially if combined with radiotherapy)

Question 2

3 CD’s of B cells for immunophenotyping

Answer 2

CD20, 45, 79a

Question 3

3 CD’s of T cells for immunophenotyping

Answer 3

CD3, 7, 45

Question 4

3 translocations involving CBF that act as dominant inhibitors of CBF

Answer 4

ETO/AML1, CBFB-MYH11, TEL/AML1

Question 5

Abnormal pop of cells, 60% of total, Co-expressing CD34 and CD33 =

Answer 5

AML

Question 6

AML is rapidly proliferative clones of what lineages

Answer 6

Myeloid, Erythroid, Meg

Question 7

Auer Rods

Answer 7

Needle-like, eosinophilic crystals formed by proteins normally found in secondary granules of granulocytes

Question 8

Auer Rods are always dx of

Answer 8

Myeloid Blasts

Question 9

Best way to gauge a patient’s iron stores

Answer 9

Look directly at what’s in the marrow

Question 10

CD33 is a marker of

Answer 10

Granulocytes

Question 11

CD34 is a marker of

Answer 11

hematopoietic stem cells

Question 12

Chronically proliferating clones which differentiate to circulating blood cells

Answer 12

Myeloproliferative Disease

Question 13

Easy morphologic tipoff for blast cell ID

Answer 13

Auer Rods

Question 14

EBV is associated with almost all cases of

Answer 14

endemic (African) Burkitt Lymphoma and post-transplant lymphoproliferative disease (and proportion of pts w/ Hodgkin lymphoma)

Question 15

Etoposide is associated with risk of development of

Answer 15

secondary leukemias associated with balanced translocations

Question 16

Flow cytometry doesn’t work well for ___ cells

Answer 16

unusually large

Question 17

Forward scatter is proportional to

Answer 17

Size

Question 18

Gold standard definition of blast

Answer 18

Appearance similar to undifferentiated hematologic precursor cell

Question 19

H Pylori has been implicated in pathogenesis of

Answer 19

Gastric mucosa B cell (MALT) lymphoma

Question 20

HIV infection is associated with increased incidence of what cancer

Answer 20

lymphomas at unusual sites such as CNS

Question 21

HIV-associated lymphomas are usually of

Answer 21

B cell origin and of high-grade histology

Question 22

How does flow for light chains on b cells help distinguish them

Answer 22

Tumor b cells express only 1 (k or g), while normal polyclonal population will express both

Question 23

lymphoid blasts outside marrow =

Answer 23

Lymphoblastic Lymphoma

Question 24

Morphologic features that suggest a leukocyte is a blast?

Answer 24

(1) Large; (2) High nuclear:cyto; (3) Prominent nucleolus; (4) Immature chromatin (faint/smudgy); (5) Appearance shared by many cells on slide

Question 25

Most important morphological feature of blast

Answer 25

Appearance shared by many cells on slide (cookie cutter)

Question 26

Mutations in NPM1 and FLT3 are common in (AML) pts with an

Answer 26

intermediate cytogenetic risk

Question 27

Myeloblasts proliferating outside bone marrow and peripheral blood are called

Answer 27

Myeloid Sarcoma

Question 28

myeloid blasts outside marrow =

Answer 28

Myeloid Sarcoma

Question 29

Myelopthisic =

Answer 29

Leuko-erythroblastic

Question 30

Needle-like, eosinophilic crystals formed by proteins normally found in secondary granules of granulocytes

Answer 30

Auer Rods

Question 31

Pathogen associated with gastric mucosa b cell lymphoma

Answer 31

H Pylori

Question 32

Ph translocation

Answer 32

9; 22

Question 33

Poorly Functioning Clones =

Answer 33

Myelodysplastic Syndrome

Question 34

Prevalance of TEL-AML1 translocation

Answer 34

10% of newborns (1 in 100 go on to develop ALL)

Question 35

Reference myeloid to erythroid ratio

Answer 35

2:1 to 5:1

Question 36

Sequenced based date for what 3 oncogenes is requred for AML w/ normal cytogenetics

Answer 36

NMP1, FLT3, CEBPA

Question 37

Side scatter is high for cell with

Answer 37

Internal granules or segmented nuclei

Question 38

t (11; 14) =

Answer 38

Mantle Cell Lymphoma (very specific)

Question 39

t (12; 21) =

Answer 39

TEL/AML1 --> B-ALL

Question 40

t (14; 18) =

Answer 40

Overexpression of BCL-2 (follicular lymphoma)

Question 41

t (15; 17) =

Answer 41

PML-RARA

Question 42

t (8; 21) =

Answer 42

ETO/AML1 --> AML

Question 43

t (9; 22) =

Answer 43

BCR-ABL1 --> CML

Question 44

T/F: Flow cytometry can tell you what fraction of bone marrow is made up by blasts

Answer 44

False: Pathologist count

Question 45

T/F: Gene amplification is not a common feature in hematopoietic malignancy

Answer 45

TRUE

Question 46

T/F: When malignant cells have telomerase, it was probably an initiating factor

Answer 46

False, probably a consequence

Question 47

Tier 1 mutation

Answer 47

AA coding region of annotated exons; Consensu pre-mRNA splice-site regions; RNA genes (including microRNA)

Question 48

Tier 1 mutations in AML vs other malignancies

Answer 48

Fewer tier 1 mutations

Question 49

Tier 2 mutation

Answer 49

Highly conserved regions of genome; known or suspected reg element

Question 50

Tier 3 mutation

Answer 50

Nonrepetitive regions not meeting criteria for 1 or 2

Question 51

Tier 4 mutation

Answer 51

repeptivie elements; noncomplex regions

Question 52

What is always dx of Myeloid Blasts

Answer 52

Auer Rods

Question 53

When should you suspect a hematologic malignancy?

Answer 53

(1) When bone marrow not functioning normally, and you can't find a simpler explanation; (2) When lymphatic tissues are enlarged, infectious etiology can't be found