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IBI Test 3 > B3.041 - Pre-work Intro to Lymphoid Neoplasms > Flashcards

B3.041 - Pre-work Intro to Lymphoid Neoplasms Flashcards

1
Q

Where can lymphoid neoplasms be derived from

A

Precursor cells (B or T ALL/LBL) Mature B cells Mature T/NK cells

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2
Q

What is are the 3 possible presentations of lymphoid neoplasms

A

primarily in: Bone marrow and blood Lymph nodes extranodal tissues

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3
Q

Classification of lymphoid neoplasms are based on what

A

cell of origin Developmental stage of transfromed cell clinical, immunophenotypic, genetic and molecular findngs

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4
Q

Classification of lymphoid neoplasms doe snot distinguis what

A

if its primarily blood presentation or lymph presentation

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5
Q

What is the primary involvement of leukemia’s

A

Bone marrow or blood

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6
Q

What is the origin of leukemia’s

A

Myeloid or lymphoid

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7
Q

What might leukemia’s secondarily involve

A

Lymph nodes and solid tissues

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8
Q

What is the tissue involvement of lymphomas

A

Lymph node or solid tissue

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9
Q

What is the origin of lymphomas

A

Lymphoid

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10
Q

What might lymphomas secondarily involve

A

Bone marrow and blood

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11
Q

Chronic lymphocytic leukemia aka

A

Small lymphocytic lymphoma

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12
Q

Acute lymphoblastic leukemia aka

A

Lymphoblastic lymphoma

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13
Q

Acute lymphoblastic leukemia aka

A

Lymphoblastic lymphoma

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14
Q

What does acute mean with leukemia’s

A

Primarily immature cells (blasts) Untreated has a rapidly progressive track and is fatal in weeks to months

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15
Q

What does chronic mean in regards to leukemia’s

A

Primarily mature or maturing cells Untreated slowly progressive and fatal in months to years

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16
Q

About 80% of all lymphomas are derived from what

A

B Cells

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17
Q

How much of lymphomas are derived from T or NK cells

A

20%

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18
Q

What is the most common extranodal mature T neoplasm located in

A

Skin

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19
Q

What is the most common skin lymphoma

A

Mycosis fungoides

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20
Q

What are the markers of mature B cell neoplasms

A

CD19, CD20

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21
Q

What do mature T cell neoplasms express

A

CD2 CD3 CD5 CD7 CD4 CD8 (variable)

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22
Q

Any given B cell neoplasm will express what

A

The same Igs, meaning they’re monoclonal

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23
Q

What does monoclonal mean

A

All these cells with have a kappa light chain or a lambda light chain but not both

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24
Q

What does monoclonal surface Ig mean clinically

A

You can distinguish between monoclonal neoplasms from non neoplastic responses

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25
Q

Which CDs do not help to distinguish clonality

A

CD4 and CD8

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26
Q

Describe the genetics of mature T cell neoplasms

A

Monoclonal rearranged T cell receptor genes

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27
Q

Describe the genetics of mature B cell neoplasms

A

Monoclonal rearranged immunoglobulin genes

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28
Q

In mature B neoplasms there are often translocations involving what

A

Chromosome 14

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29
Q

Why do translocations to chromosome 14 often result in cancer?

A

Its the IgH gene locus which is always on

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30
Q

Describe lymph node architecture

A

Diffuse Modular Follicular

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31
Q

Describe small lymphoid cell size features

A

Tend to be more indolent Immunophenotype and genetics re critical for diagnosis and classification

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32
Q

Describe characteristics of medium-large lymphoid

A

Tend to be more aggressive Morphology supplemented by immunophenotype and genetics for diagnosis and classification

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33
Q

What are the most common B cell neoplasms

A

CLL/SLL

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34
Q

What are the clinical features of CLL and SLL

A

Older adults, most common leukemia in US variable anemia/thrombocytopenia Lymphadenopathy Immunologic abnormalities

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35
Q

what is the morphology of CLL

A

small mature lymphocytes in blood, bone marrow

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36
Q

What is the morphology of SLL

A

diffuse infiltrate of small lymphocytes in lymph nodes

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37
Q

What is the characteristic immunophenotype of CLL/SLL

A

CD19+, CD20+, CD5+ CD10- slg+(clonal)

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38
Q

What is characteristic of mutated CLL/SLL

A

Somatic hypermutation in IgVh (>2% variance from germline sequence 40-50% of cases)

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39
Q

what other lymphoma shares these immunophenotypes

A

mantle cell lymphoma

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40
Q

what is the favorable type of CLL/SLL

A

Mutated

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41
Q

what is unmutated CLL/SLL

A

no somatic hypermutation in IgVh

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42
Q

what are other genetic abnormalities with CLL/SLL

A

Deletion 13q, deletion 11q, deletion 17p, trisomy 12

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43
Q

which other genetic abnormality in CLL/SLL is considered favorable

A

deletion 13q

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44
Q

what is the median survival of CLL/SLL

A

6 years

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45
Q

why is the median survival of CLL/SLL misleading

A

its 3 y for unmutated and over 7 for mutated

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46
Q

what is hairy cell leukemia clinically seen in

A

middle aged older adults

47
Q

what are clinical features of hairy cell leukemia

A

splenomegaly hepatomegaly pancytopenia infections

48
Q

What is the morphology of hairy cell leukemia

A

lymphocytes with villous projections

49
Q

what is the immnophenotype of hair cell leukemia

A

CD19+, CD20+, CD11c+ slg+ (Clonal) CD25+ CD103+ annexin A1 CD10-, CD5-

50
Q

what are the genes associated with hairy cell leukemia

A

BRAF mutation

51
Q

which immunophenotypes are specific for hairy cell leukemia

A

CD103 and Annexin A1

52
Q

What are clinical feature of B cell neoplasm follicular lymphomas

A

Middle aged and older adults, common lymphadenopathy hepatosplenomegaly

53
Q

What is the morphology of follicular lymphoma

A

nodular infiltrate of small cleaved lymphocytes in lymph nodes diminished mantle zone; absent tingible body macrophages

54
Q

what is the immunophenotype of follicular lymphoma

A

CD19+, CD20+, CD10+, CD5-, slg+ (clonal)

55
Q

what is a gene mutation associated with follicular lymphoma

A

t(14;18) - overexpression of Bcl-2 protein prevents apoptosis

56
Q

what are clinical features of B cell neoplasm mantle cell lymphoma

A

middle aged and older adults, uncommon lymphadenopathy, hepatosplenomegaly, often adnvanced stage

57
Q

what is the morphology of mantle cell lymphoma

A

nodular or diffuse infiltrate of small lymphocytes in lymph nodes

58
Q

what is the immunophenotype of mantle cell lymphoma

A

CD19+, CD20+, CD10-, CD5+, slg+ (clonal) Cyclne D1+

59
Q

what is a gene mutation associated with mantle cell lymphoma

A

t(11;14) - overexpression of cyclin D1

60
Q

what is the course and prognosis of mantle cell lymphoma

A

3-4 y poor prognosis

61
Q

what is a main difference between CLL/SLL and mantle cell lymphoma given they share many of the same immunophenotypes

A

CLL/SLL is very indolent Mantle cell is very aggressive

62
Q

how do you distinguish between CLL/SLL and mantle cell

A

look for the genetic mutation of t(11;14)

63
Q

what are the clinical feature of B cell neoplasms marginal zone lymphoma

A

Extranodal - GI, lung, skin,eye, head and neck Association with immune reactions and chronic infection

64
Q

what is the morphology of marginal zone lymphoma

A

infiltrate of the lymphoid cells into epithelium and expanded marginal zone

65
Q

what is the immunophenotype of marginal zone lymphoma

A

CD19+, CD20+, CD10-, CD5-, slg+ (clonal)

66
Q

early on in the disease if its being caused by chronic infection how can you treat it

A

antibacterials

67
Q

what are clinical features of B cell neoplasm diffuse large B cell lymphoma

A

middle aged and older adults lymphadenopathy hepatosplenomegaly frequently extranodal

68
Q

what is the morphology of diffuse large B cell lymphoma

A

diffuse infiltrate of large lymphocytes in lymph nodes

69
Q

what is the immunophenotype of diffuse large B cell lymphoma

A

CD19+, CD20+, CD10+/-, CD5-, slg+ (Clonal) some types associated with EBV or HHV8

70
Q

what are genetic mutations associated with diffuse large B cell lymphoma

A

Variable, may be positive for MYC translocation

71
Q

what is significant about diffuse large B cell lymphomas with a MYC transolaction

A

They are more aggressive

72
Q

what are burkitt lymphoma clinical features

A

Rapidly progressive tumor mass extremely high growth fraction tumor lysis syndrome common when treatment initiated

73
Q

where is burkitt lymphoma endemic to and who does it affect

A

equatorial africa children mostly mostly associated with EBV

74
Q

what form of burkitt lymphoma is common in US, who does it affect and what is it associated with

A

sporadic form, children and adults, EBV

75
Q

what other disease is burkitt lymphoma associated with

A

HIV

76
Q

nearly all of the cancer cells in burkitt lymphoma are constantly dividing, what does this mean clinically

A

it grows very fast and is very responsive to treatment tumor lysis syndrome is an issue

77
Q

what do you give to patients to prevent tumor lysis syndrome

A

give lots of fluids and kidney protection

78
Q

which from of burkitt lymphoma does the best in respose to treatment

A

endemic form

79
Q

which type of burkitt lymphoma does the worst in response to treatment

A

HIV associated

80
Q

what is the morphology of burkitt lymphoma

A

diffuse infiltrate of medium sized lymphocytes in lymph nodes high mitotic rate; tingible body macrophages (“Starry sky”)

81
Q

what is the immunophenotype of burkitt lymphoma

A

CD19+, CD20+, CD10+, CD5-, slg+ (clonal), TdT-

82
Q

what are genetic mutations relevant to burkitt lymphoma

A

t(8;14) [variants t(2;8) or t(8;22)] - MYC oncogene translocated to IgH locus and constitutively expressed

83
Q

if you see starry sky what should you think

A

burkitt lymphoma

84
Q

how do you distinguish between burkitt lymphoma and follicular lymphoma

A

the morphology is different

85
Q

what does TdT- tell you for diagnosis

A

its burkitts lymphoma and not ALL

86
Q

is the MYC mutation unique to burkitt lymphoma

A

no

87
Q

which type of lymphoma has an almost 100% mitotic fraction

A

Burkitt

88
Q

what is mycosis fungoides/sezary syndrome

A

T cell neoplasm of the skin that can go into the blood

89
Q

what are clinical feature of mycosis fungoides

A

older adults cutaneous patch, plaque and nodule stages lymphadenopathy unusual

90
Q

what is the morphology of mycosis fungoides

A

MF - dermal iniltrate of atypical lymphocytes with epidermotropism and pautrier microabscesses SS - circulating lymphoid cells with cerebrioform nuclei and eryhroderma

91
Q

what is the immunophenotype of mycosis fungoides

A

CD4+

92
Q

what is the course and prognosis of mycosis fungoides

A

indolent, chronic course with 90% 5 year survivial

93
Q

what are clinical features of adult T cell leukemia/lymphoma

A

endemic to japan, caribbena, west africa middle aged to older adults caused by HTLV-I generalized lymphadenopathy, skin lesions, hepatosplenomegaly, lymphocytosis, hypercalcemia

94
Q

what infection causes adult .T cell leukemia

A

HTLV-1

95
Q

what are clinical features of T cell neoplasm anaplastic large cell lymphoma

A

lymphadenopathy

96
Q

what is the immunophenotype of anaplastic large cell lymphoma

A

CD30+

97
Q

what are the genetics of anaplastic large cell lymphoma

A

t(2;5) - rearrangement of ALK

98
Q

what indicates a poor prognosis for anaplastic large cell lymphoma

A

ALK-

99
Q

what are clinical feature of peripheral T cell lymphoma

A

lymphadenopathy fever, pruritus, weight loss eosinphilia

100
Q

what is course and prognosis of peripheral T cell lymphoma

A

poor prognosis with 20-30% 5 year survival

101
Q

what are clinical features of hodgkin lymphoma

A

bimodal age distribution (15-35 y and >50) most common malignancy in 10-30 age group in US lymphadenopathy, splenomegaly constitutional B symtoms Immune dysfunciton possible relation to EBV infection

102
Q

what is the morphology of classical hodgkin lymphoma

A

Reed-Sternberg cells in a polymorphous cellular backgroudn

103
Q

What is the morphology of nodular lymphocyte predominant hodgkin lymphoma

A

No Reed-sternberg cells; large B cells in a background of small lymphocytes

104
Q

what are constitutional B symptoms

A

fever night sweats weight loss

105
Q

what are characteristics specific to hodgkin lymphoma

A

Hodkin - Heterogenous cell population Reed sternberg cells Primary extranodal presentation rare Often localized to a single nodal group spread along contiguous nodal groups Waldeyer ring and mesenteric nodes rarely involved young adults an dedlerly Good prognosis

106
Q

what are characteristics specific to non hodgkin lymphoma

A

homogenous cell population no reed sternberg cells Primary extranodal presentation common Frequently involve multiple nodal groups Spred to any site in an unpredictable manner Waldeyer ring and mesenteric nodes commonly involved Any age variable prognosis

107
Q

what is the immunophenotype of HL

A

Reed sternberg cells CD15+, CD30+. CD20 +/-, neg for most other B and T cell markers

108
Q

what is a genetic mutation associated with HL

A

mutated germinal center B cells that escape apoptosis

109
Q

what is course and prognosis of HL

A

excellent at early stage Risk of second malignancy in long term survivors

110
Q

Describe CD20 status on all B cell neoplasms with small lymphocytes

A

+ or all

111
Q

Describe CD5 status for all B cell neoplasms with small lymphocytes

A

+ for CLL/SLL, Mantle cell - for Hairy cell, MALT, follicular

112
Q

Describe the CD10 status of all B cell neoplasms with small lymphocytes

A

+ for Follicular, - for the rest

113
Q

Non neoplastic proliferations of lymphocytes

A

viral infections Acute bacterial infections chronic bacterial infections atypical lymphocytes

114
Q

non neoplastic lymphadenopathy

A

follicular hyperplasia due to infections Paracortical hyperplasia due to viral infections/drugs Sinus histiocytosis - lymph nodes draining infection

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