WBCII, Putthoff, lec Flashcards

1
Q

What are the neoplasms of immature B and T cells

A

ALL from B cell or T cell

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

what is the most common childhood cancer

A

ALL, 85% B cell

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

where is precursor for B and T cell ALL

A

B from bone marrow

T from tymus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

peak incidence of B and T cell ALL

A

B is age 3

T is adolescence when thymus is full size

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

what genotypes are assoc with B cell ALL

A

t(12;21) involves RUNX1 and ETV6
trisomy 4 7 and 10
philedelphia chromosome 9;22
hyperdiploidy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

what genotypes are assoc with T cell ALL

A

NOTCH1 mutations

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What are clinical features of ALL

A

abrupt onset Sx
fatigue, fever, infections, bleeding
bone pain, lymphadenopathy, HSM, testicular enlargement
HA, vomiting, nerve palsies from meningeal involvemnt

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

what are the Sx specific to T cell ALL

A

complications from compression Large vessels and airways in the mediastinum

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What Sx of ALL are from problems assoc with marrow

A

fatigue, fever, infecitons, bleeding

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

what Sx of ALL are assoc with neoplastic infiltration

A

bone pain
adenopathy
HSM
testicular involvement and the CNS Sx

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is distribution in poplation of ALL

A

more common in hispanics
3W:B
more male than female

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

what are good prognostic factors for ALL

A

between ages 2-10, low WBC, hyperdiploidy, trisomies 4,7,10 and t12;21

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

what are bad prognostic factors of ALL

A

age less than 2 or greater than 10
peripheral blast count >100,000
the philadelphia translocation 9;22 BCR/-ABL fusion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

what do the nuclei look like on smear of ALL

A

spiculated nuclei

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What CD marker is assoc with B cell neoplasms

A

CD 10

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

increased intracell terminal dinucleotide transferase TdT is assoc with what neoplasms

A

B and T cell ALL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

most common leukemia in adults in westerm world?

A

CLL or SLL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What genotypes are assoc with CLL

A

trisomy 12

deletions:11q,13q,17p

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What is immunophenotype CLL

A

CD19, CD20, CD23, CD5

low level Ig on surface

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

population affected by CLL

A

2M:1F

avg age 60

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Sx CLL

A

easy fatiguability, weight loss, anorexia
generalized lymphadenopathy, HSM
increased infections
hypogammaglobulinemia

sometimes hemolytic anemia or thrombocytopenia from auto Ab

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

buzzwords for CLL

A

smudge cells, growth confined to proliferation centers, richter syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

what is richter syndrome

A

the transformation of CLL to diffuse large B cell lymphoma or a prolymphocyte evolution
aggressive with less than 1 yr survivial

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What is absolute lymphocyte count in CLL

A

> 5000

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

how do you Dx CLL

A

peripheral blood smear

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Prognosis CLL

A

4-6 yrs. can be >10 if mild

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Tx CLL

A

gentle chemo and immunotherapy with Ab to CD20 or inhibitors of BTK

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

What is the most common indolent NHL in US

A

follicular lymphoma

29
Q

Where are follicular lymphomas from

A

germinal center B cells

30
Q

genotypes assoc with follicular lymphoma

A

t14;18 which causes the BCL2-IgH fusion that causes overexpression and increased inhibition of apoptosis

31
Q

population affected by follicular lymphomas

A

middle age males

rare in Asia

32
Q

What is histo characteristics of follicular lymphomas

A

no apoptotic cells, follicular cells everywehre
increased centrocytes and centroblasts
lymphocytosis <20,000

33
Q

Immunophenotype follicular lymphoma

A

CD 19, CD 20, CD10, surface Ig, BCL6!

34
Q

what are Sx of follicular lymphoma

A

generalized painless lymphadenopathy

extranodal sites like GI CNS and testis is rare

35
Q

Prognosis of follicular lymphoma

A

7-9 yrs

36
Q

Tx follicular lymphoma

A

mild chemo, mainly palliative

37
Q

Transofrmation of follicular lymphoma

A

diffuse large B cell lymphoma and tymor that resembles Burkitt lymphoma

38
Q

mean survival if follicular lymphoma transforms

A

less than 1yr

39
Q

what is the most common non hodgkin lymphoma

A

diffuse large B cell lymphoma

40
Q

where are diffuse large B cell lymphomas derived from

A

germinal and post germinal

41
Q

what mutations are assoc with diffuse large B cell lymphomas

A

BCL6 dysregulation chrom 3q27

42
Q

how are the BCL2 dysregulation of diffuse large B cell differentiated from follicular lyphomas

A

there is absolutely no follicular formation

43
Q

What are the subsets of diffuse large cell B lymphoma

A
immunodeficiency associated (EBV)
primary pleural effusion lymphoma (KSHV/HHV8)
44
Q

what will a slide looks like of large diffuse B cell lymphoma

A

large cell size with diffuse pattern of growth
round/oval nucleus that looks vesicular
nucleoli present
abundant cytoplasm

45
Q

immunophenotype of large diffuse b cell lymphoma

A

CD19 and 20
CD 10 and BCL6
most with surface Ig

46
Q

what is the clinical presentation of diffuse large b cell lymphoma

A

rapidly enlarging mass at nodal and extranodal sites

47
Q

where are common sites of diffuse large B cell lymphoma

A

waldeyer ring
oropharyngeal lymph
GI, skin, bones, brain

48
Q

Prognosis of diffuse large b cell lymphoma

A

rapidly fatal without Tx

49
Q

Tx diffuse large B cell lymphoma

A

aggressive chemo with Ab CD20

50
Q

age and sex affected by diffuse large b cell lymphoma

A

male
age 60
can occur in childhood and adolescence

51
Q

Where are the cells derived from in burkitt lymphoma

A

germinal center B cells

52
Q

mutations associated with burkitts lymphoma

A

t8;14

MYC and IgH or even kappa and gamma light chains

53
Q

What is the role of the MYC gene

A

increases expression of gene for aerobic glycolysis “warburg effect”

54
Q

What is the subset of burkitt lymphoma

A

EBV associated

55
Q

what are the categories of burkitt lymphoma

A

african (endemic)
sporadic (nonendemic)
HIV (aggressive)

56
Q

which category of burkitt lymphoma has a mass that invades mandible commonly

A

endemic (african)

57
Q

which category of burkitt lymphoma has a mass that invades ileocecum and peritoneum commonly

A

sporadic (nonendemic)

58
Q

what is distribution of burkitt lymphoma

A

30% NHL childhood tumors

any age really

59
Q

what is the fastest growing human tumor

A

burkitt lymphoma

60
Q

what will histo look like of burkitt lymphoma

A
diffuse infiltrate of med size lymphoid cells
several nucleoli, coarse chromatin
high mitotic index
many apoptotic cells
"starry sky" phagocytes
61
Q

what will histo of burkitt taken from bone marrow look like

A

nucleoli with royal blue cytoplasm with clear vacuoles

62
Q

What does starry sky phagocytes look like

A

abundant clear cytoplasm

63
Q

what is the immunophenotype of burkitt lymphoma

A

IgM,CD19

CD20 CD10 BCL6

64
Q

what surface marker is not expressed on burkitt even though it is a germinal tumor

A

BCL2

65
Q

Tx burkitt lymphoma? prognosis

A

intensive chemo

most children cured, adults not as much

66
Q

class II MHC molecules are expressed on what

A

macrophages, B lymphocytes and dendritic cells

67
Q

role of B lymphocyte

A

neutralization of microbe, phagocytosis and C’ activation

68
Q

roles of helper T cells

A

activation of macrophages
inflammation
activation of T and B cells

69
Q

how do T cells rapidly enter parenchyma & become memory

A

high endothelial venule