lymphoma I and II Flashcards

1
Q

what is staging?

A
  • looking for where the tumor is

- this is done in hodgkin and non-hodgkin lymphoma

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2
Q

some tests done in staging…

A

Labs: cbc, lytes, creatinin, BUN, LFTs
LDH: can be elevated in non-hodgkins
CT/PET: abd, pelvis, chest
BMB: bilateral

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3
Q

stage definitions I-IV

A

I - single lymph node
II - two or more lymph nodes on same side of diaphragm
III - two or more lymph nodes on different sides of diaphragm
IV - either multifocal involvement with one or more extra lymphatic site +/- associated lymph nodes OR involvement of extra lymphatic organ with distant nodal involvement

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4
Q

staging subclassifications

A

A - asymptomatic

B - either night sweats, fever, or greater than 10% weight loss in 6 months

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5
Q

what is meant by extra-lymphatic

A

tissue other than lymph nodes, thymus, spleen, Waldeyer’s ring, appendix, and Peyer’s patches

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6
Q

list some indolent lymphomas

A
  • CLL
  • follicular lymphoma
  • marginal zone lymphoma
  • MALT
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7
Q

list some aggressive lymphomas

A
  • ALL

- Burkitt’s lymphoma

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8
Q

list some intermediately aggressive lymphomas

A
  • diffuse large B cell lymphoma
  • peripheral T cell lymphoma
  • mantle cell lymphoma
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9
Q

DLBCL who gets it?

A

adults and children, but mostly adults, median 64

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10
Q

DLBCL curability and percent presence in bone marrow?

A
  • curability 30-40%

- BM 15-20%

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11
Q

DLBCL benign equivalent

A

large replicating cells of germinal center and paracortex

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12
Q

DLBCL pathophysiology

A

diffuse infiltration of lymph node, increased mitotic rate, necrosis may be present

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13
Q

DLBCL markers

A
  • CD19+, CD20+
  • possible: bcl-6 and/or bcl-2 expression
  • rearrangement of IgH, IgL chains
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14
Q

DLBCL subtypes of germinal center B cell origin, and which is better?

A

GCB - germinal center B cells (better survival)

ABC - activated B cell

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15
Q

characters of newly discovered highly aggressive DLBCL

A

over expression of “myc” oncogene and bcd-2

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16
Q

DLBCL five point scale and rating

A

1) over 60
2) stage III or IV
3) elevated LDH
4) poor performance status
5) extra nodal involvement

0-1 - low
2 - low intermediate
3 - high intermediate
4-5 - high risk

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17
Q

DLBCL treatment

A
  • R-CHOP
  • usually chemo as well
  • autologous transplant only in refractory cases
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18
Q

what is R-CHOP?

A
  • rituximab
  • cyclophosphamide
  • adriamycin (anthracycline backbone)
  • vincristine
  • prednisone
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19
Q

rituximab is anti-CD….

A

CD20

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20
Q

what percent of NHL is follicular lymphoma?

A

22%

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21
Q

who gets FL?

A

adults over 40, median 59

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22
Q

FL grades and aggressiveness

A

grade I - 5 or less large cells in follicle
grade II - 6-15 large cells
grade III - more than 15

grades I/II are considered indolent while grade III is treated as an intermediate large cell lymphoma

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23
Q

FL presents at what stage?

A

III or IV

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24
Q

FL life expectancy

A

7-10 years but highly variable because there is an aggressive type

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25
Q

FL benign equivalent

A

small cleaved cell of germinal center

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26
Q

FL cell characteristics

A
  • clumped chromatin
  • infrequent nucleolus
  • like small lymphocyte but irregular nuclear profile with nuclear folds
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27
Q

FL follicle characteristics

A
  • maintains normal structure, but monotonous proliferation of one cell type
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28
Q

FL chromosomal translocation

A
  • t(14;18)

- upregulates bcl-2 which is anti-apoptosis

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29
Q

FL immunophenotype

A

positive: CD19, CD20, CD10, bcl-2
negative: CD5, cyclin D1

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30
Q

clinical course of FL

A

indolent - slow growing with relapses

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31
Q

FLIPI scale

A

five point scale

1) over 60yo
2) elevated LDH
3) stage III or IV
4) greater than 4 LN sites
5) hemoglobin less than 12

3-5 - high
2 - intermediate
0-1 - low

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32
Q

FL - reasons to move from watchful waiting to treatment

A

1) Bulky, painful adenopathy
2) Organ involvement or impairment
3) B symptoms (severe)
4) Cytopenias due to bone marrow involvement
5) Autoimmune cytopenias (Idiopathic Thrombocytopenic Purpura; Autoimmune Hemolytic Anemia)
6) High FLIPI score

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33
Q

FL treatment

A

stages I and II - radiation alone is usually sufficient

stages III and IV - gentle chemo including rituximab (R-CVP)

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34
Q

MZL aggression?

A

indolent

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35
Q

three subtypes of MZL

A

1) extranodal marginal zone B cell lymphoma (MALT)
2) nodal marginal zone
3) splenic marginal zone lymphoma

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36
Q

MALT MZL can be in the….

A

GI tract, tear duct, salivary gland, etc

37
Q

splenic MZL can be in the

A

spleen or bone marrow

38
Q

MZL treatment

A

same as FL
stages I and II - radiation alone is usually sufficient
stages III and IV - gentle chemo including rituximab

39
Q

MZL different from other indolent lymphomas because…

A

tends to be curable

40
Q

MZL cell of origin

A

post germinal memory B cell that has the capacity to become a marginal zone cell.

41
Q

MZL cell markers

A

positive: CD19, CD20
negative: CD5, CD10, cyclin D1

42
Q

gastric MALT lymphoma symptoms similar to…

A

peptic ulcer

43
Q

gastric MALT lymphoma is driven by…which can lead to which translocation?

A
  • H. pylori

- t(11;18) which can produce a more aggressive MALT lymphoma

44
Q

gastric MALT treatment

A

if H. pylori positive: antibiotics

if H. pylori negative: radiation therapy or chemotherapy is necessary. Rituxan has also been proven to be beneficial

45
Q

hairy cell leukemia aggressiveness

A

indolent

46
Q

why HCL called a leukemia?

A

it’s in the blood and bone marrow but derived from a lymphocyte

47
Q

HCL cell markers

A

positive: CD19, CD20
negative: CD5, CD10

48
Q

HCL visualization

A
  • “hairy” cytoplasmic projections

- in bone marrow, fried egg cytoplasm

49
Q

HCL treatment

A
  • purine analogue such as cladribine

- rituximab in relapse

50
Q

Burkitt’s lymphoma agressiveness

A

highly aggressive

51
Q

who gets Burkitt’s?

A

children, young adults, and immunocompromised

52
Q

three types of Burkitt’s

A

1) endemic - africa, EBV, children’s jaws
2) immunocompromised - HIV/AIDS, 30% EBV positive, abdominal masses, marrow and nodal involvement, higher in cases with higher CD4 counts
3) sporadic - same as immunocomromised but in healthy children and young adults

53
Q

Burkitt’s benign equivalent

A

small non-cleaved cell of germinal center

54
Q

Burkitt’s pattern under a microscope

A
  • starry sky
  • because of macrophages attracted by ineffective proliferation, want to phagocytose
  • this is all due to high mitotic rate with high failure
55
Q

Burkitt’s chromosomal translocation

A
  • t(8;14) - causes up regulation of “myc” oncogene

- myc is major “on switch” of cell cycle and causes rapid proliferation

56
Q

how long is survival with Burkitt’s without treatment?

A

just a couples weeks

57
Q

Burkitt’s treatment

A

aggressive multi-drug chemo for 6-8 months

58
Q

who gets mantle cell lymphoma?

A

adults, mean age 63

59
Q

mantle cell lymphoma aggressiveness

A

appears indolent but acts intermediate to aggressive

60
Q

mantle cell benign equivalent

A

lymphocyte of inner mantle zone

61
Q

mantle cell pathology

A

t(11;14) upregulates bcl-1, increases cyclin D1 production

62
Q

treatment of mantle cell

A
  • poor response to therapies
  • 5 year survival is 30-50%
  • allogeneic and autologous transplants can be helpful
63
Q

commonality of T cell lymphoma

A

only 20% of non-hodgkin lymphoma

64
Q

where are most T cell lymphomas?

A

extranodal

65
Q

if T cell lymphoma is nodal……

A

very bad, worse than any B cell lymphoma

66
Q

common skin T cell lymphoma

A

mycosis fungoides

67
Q

anaplastic large cell lymphoma is what type of lymphoma?

A

T cell

68
Q

what is the translocation and protein in anapestic large cell lymphoma?

A

t(2;5) - ALK1

69
Q

ALK1 vs non-ALK1 lymphoma

A

ALK1 positive has 93% survival rate while ALK1 negative has only 37% survival

70
Q

3 basic characteristics of Hodgkin lymphoma

A
  • less common than non-hodgkin
  • bimodal (adolescence and then again 6th decade)
  • spreads contiguously in lymphatics and doesn’t often skip a region
71
Q

what diagnoses hodgkin lymphoma?

A

reed sternberg cell, which is an altered B cell

72
Q

hodgkin cell indicators

A

positive: CD15, CD30
negative: CD45

73
Q

virus sometimes associated with hodgkin

A

EBV

74
Q

4 subtypes of classical hodgkin lymphoma

A

1) nodular sclerosing
2) lymphocyte rich
3) lymphocyte depleted
4) mixed cellularity

75
Q

nodular sclerosing classical hodgkin characteristics

A
  • most common
  • neck and anterior mediastinal adenopathy in young females
  • fibrotic capsule and bands of subdividing tissue
  • lacunar varient reed-sternberg cells
76
Q

lymphocyte rich classical hodgkin characteristics

A
  • limited disease in neck of young adults

- “L and H” or “popcorn” RS cells

77
Q

lymphocyte depleted classical hodgkin characteristics

A
  • often retroperitoneal
  • aggressive
  • pleomorphic RS cells
78
Q

mixed cellularity classical hodgkin characteristics

A
  • aggressive
  • older patients
  • mononuclear RS cells - more of them
79
Q

crude disease activity measure in hodgkins

A

ESR

80
Q

hodgkin overall cure rate

A

over 80%

81
Q

hodgkin treatment of stages I and II

A

abbreviated chemo and radiation, in stage one radiation alone may suffice

82
Q

hodgkin treatment of stages III and IV

A

ABVD x 6, anthracycline backbone

83
Q

what is considered primary refractory disease in hodgkin and what is the prognosis?

A
  • grows through therapy or relapses in less than six months

- aggressive and often fatal

84
Q

three stages of mycosis fungoides

A

three stage process of mycosis fungoides

1) patch
2) plaque
3) tumor

85
Q

mycosis fungoides tumor stage

A
  • mushroom shaped
  • predominant in groin, face, axilla, body folds
  • generalized lymphadenopathy
  • ulceration or necrosis
86
Q

50% of those with CTCL die from….

A

staph or pseudomonas

87
Q

sezary syndrome triad

A

1) erythroderma
2) leukemia (defining characteristic)
3) lymphadenopathy

88
Q

sezary cells in peripheral blood

A
  • large cells
  • cleaved nuclei
  • scant cytoplasm