Path/Heme Maligs III

Question 1

Name some Ig promoters

Answer 1

IgH (14q32)
Ig lambda (22q11)
Ig kappa (2p12)

Question 2

Bcl2 and Bcl6 are overproduced in these neoplasms

Answer 2

Germinal center derived

Question 3

Cyclin-D1 and Myc are overproduced in these neoplasms

Answer 3

naive B cell neoplasms

Question 4

Cyclin D3 and Pax5 are overproduced in these neoplasms

Answer 4

memory B cell neoplasms

Question 5

Where does CLL primarily manifest?

Answer 5

Peripheral blood

Question 6

Where does follicular lymphoma primarily manifest?

Answer 6

Lymph nodes

Question 7

Where does Burkitt’s lymphoma primarily manifest?

Answer 7

GI tract

Question 8

CLL is derived from what type of cell?

Answer 8

Memory B cells (already class switched)

Question 9

What is a good prognosis in CLL?

Answer 9

13q del only

Question 10

What is a bad prognosis in CLL?

Answer 10

del(17p) (p53)

Question 11

Immunophenotype of CLL

Answer 11

Light chain restriction (clonal)
CD5+ (odd but characteristic)
Somatic hypermutation markers

Question 12

Two other genetic events leading to CLL

Answer 12

Trisomy 12 and del11q

Question 13

How do smudge cells help predict prognosis of CLL?

Answer 13

> 30% is good

Question 14

Morphology of CLl

Answer 14

Smudge cells
Pseudofollicular lymph nodes
Small lymphocytes with sparse cytoplasm and mature chromatin

Question 15

Common presentation of CLL

Answer 15

lymphocytosis in older males with recurrent infections (hypogammaglob)

Question 16

Common presentation of MCL and what does it mimic closely?

Answer 16

lymphocytosis in older males with recurrent infections (hypogammaglob); mimics CLL

Question 17

Where does mantle cell lymphoma primarily manifest?

Answer 17

Lymph nodes

Question 18

What is the key difference between CLL and MCL?

Answer 18

No proliferation centers in MCL

Question 19

What immunomarkers are helpful in distinguishing CLL from MCL and how so?

Answer 19

CD20+ very strong in MCL, much weaker in CLL

Question 20

What translocation is common in MCL?

Answer 20

t(11;14) aka (IgH;CyclinD1) which is ALWAYS seen by FISH

Question 21

What is the key clinical predictor of MCL?

Answer 21

Ki-67 immunostain for mitotic rate

Question 22

Where do plasma cell neoplasms primarily manifest?

Answer 22

Bone marrow

Question 23

What are some smear and lab findings of plasma cell neoplasms?

Answer 23

Rouleaux on PB; inc total protein (antibodies)

Question 24

What would you expect to see on a plasma cell neoplasm gel?

Answer 24

IgG spike

Question 25

What are two important clinical predictors of plasma cell neoplasms?

Answer 25

B2-microglobulin (bad) and del 17p (p53) - bad

Question 26

What is the common immunophenotype of plasma cell neoplasms?

Answer 26

CD38+++ CD138+++ light chain restricted

Question 27

Describe follicular lymphoma pathogenesis

Answer 27

Failure of germinal center B-cells to undergo apoptosis because they overexpress Bcl-2 due to translocation under IgH

Question 28

What are some morphological findings of follicular lymphoma?

Answer 28

No polarity No tingible body macrophages (no food!) Reduced mitotic figures (bad, normally high)

Question 29

Major immunomarker of follicular lymphoma?

Answer 29

BCL-2+

Question 30

Genetic translocation in most follicular lymphomas

Answer 30

t(14;18) in 85%

Question 31

Describe the prognostication of follicular lymphoma

Answer 31

Depends highly on grade; may progress to diffuse large B cell lymphoma

Question 32

How is follicular lymphoma graded?

Answer 32

Amount of large cells (centroblasts)

Question 33

Which is bigger, centroblasts or centrocytes?

Answer 33

Centroblasts

Question 34

Diffuse large B cell lymphoma immunophenotype

Answer 34

CD19 CD20 CD10

Question 35

Name two common translocations in diffuse large b cell lymphoma

Answer 35

t(v:3) Bcl-6 | t(14;18) Bcl2-IgH

Question 36

Most common presentation of Hodgkin's

Answer 36

Males 30-50 with adenopathy

Question 37

Morphology of Classical Hodgkin's

Answer 37

Highly variable; look for Reed-Sternberg "owl eyes" cells Diverse background of other cells +/- Nodular fibrous bands

Question 38

Morphology of Nodular lymphocyte predominant Hodgkin's

Answer 38

Smaller R-S cells with less prominent nucleoli ("Popcorn cells")

Question 39

What is a Reed-Sternberg cell?

Answer 39

Rearranged Ig genes Constitutive NFkB expression from mut or EBV Or other apoptosis mutations NO Immunoglobulin expression!

Question 40

Immunophenotype of Classical Hodgkin's

Answer 40

CD30+ (very important) CD15+ pax5+ (b cell TF)

Question 41

Key clinical predictors of Hodgkin's

Answer 41

Stage and histological type

Question 42

What is one way to differentiate between types of Hodgkin's using cell surface markers?

Answer 42

Use +/- immunoglobulins on R-S cells: + is Nodular - is classical