Lymphoreticular diseases Flashcards

1
Q

What are some 4 causes of non-neoplastic diseases of lymph nodes?

A

1) Infective (Granulomatous, Viral, Acute non-specific lymphadenitis)

2) Necrotizing (Kikuchi’s) lymphadenitis

3) Drug related lymphadenopathy

4) Autoimmune diseases (eg. RA, SLE)

5) Unknown etiology (eg. sarcoidosis)

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2
Q

What is a common histological finding in Kikuchi’s lymphadenitis?

A

Necrosis (pink area)

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3
Q

What are common histological findings in granulomatous inflammation?

A

1) Langhan’s giant cells
2) Caseous necrosis

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4
Q

What stain is used to identify Candida infections?

A

PAS (stains pseudohyphae and spores bright red)

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5
Q

What stain is used to identify PCP (Pneumocystis pneumonia)?

A

GMS (cup/boat-shaped cysts of fungi stain dark grey/black)

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6
Q

What are common histological findings in a CMV infection?

A

1) Cytoplasmic inclusions
2) Owl’s eye inclusions
3) Enlarged cells
4) Abscess presence

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7
Q

What are 3 common autoimmune causes of reactive lymphoid hyperplasia?

A

1) SLE
2) RA
3) Myasthenia gravis
4) Grave’s disease
5) Scleroderma
6) Autoimmune hemolytic anemia

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8
Q

Where do lymphomas typically manifest?

A

Outside bone marrow, at sites of normal lymphoid homing:
1) Lymph nodes
2) Spleen
3) thymus
4) MALT
5) Elsewhere (extranodal lymphomas)

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9
Q

What are 4 typical clinical presentations of lymphomas?

A

1) Enlarging masses (typically painless @ nodal tissue)

2) Pain, obstruction, perforation (compression/infiltration of hollow organs)

3) Solid organ infiltration (interference w normal organ f(x))

4) Systemic symptoms (fever, WL, night sweats)

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10
Q

How are lymphomas clinically staged?

A

Lugano classification (Ann Arbor Staging system)

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11
Q

What are the 4 stages of lymphomas? (Lugano classification)

A

Stage 1:
1 lymph node/extralymphatic site

Stage 2:
>1 lymph node regions on same side of diaphragm (± localised extralymphatic)

Stage 3:
lymph node involved on both side of diaphragm (± spleen)

Stage 4:
Diffuse extralymphatic

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12
Q

What is the tool used by clinicians to predict the prognosis of px with aggressive Non-Hodgkin’s lymphomas?

A

IPI (International Prognostic Index)

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13
Q

What are the 5 risk factors used in assessing IPI for a px with non-hodgekin’s lymphoma?

A

1) Age >60 y/o
2) Stage 3/4
3) Elevated LDH
4) Performance status
5) >1 extranodal site

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14
Q

What type of cells form Acute/Chronic Myeloid Leukemias?

A

Myeloblasts (Granulocyte precursors)

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15
Q

What type of cells form Acute Lymphocytic Leukemias?

A

Lymphoblasts (B, T, NK cell precursor)

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16
Q

What type of cells form Chronic Lymphocytic Leukemias?

A

B lymphocytes

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17
Q

What type of cells form Myelomas?

A

Plasma cells

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18
Q

What is the difference between aggressive and indolent malignant lymphomas?

A

1) Aggressive have much higher (i) proliferation and (ii) apoptotic rate

2) Aggressive have much larger proliferation margin (proliferation rate - apoptotic rate)

3) Aggressive > localised @ diagnosis (indolent > widespread)

4) Indolent currently incurable (unless localised/marrow ablation + stem cell transpact)

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19
Q

Give 3 examples of a indolent lymphoma?

A

1) Follicular lymphoma (Bcl-2)
2) MALT lymphoma
3) CLL/SLL

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20
Q

What are 3 examples of aggressive lymphomas?

A

1) Diffuse large B cell lymphoma
2) Mantle cell lymphoma
3) Burkitt lymphoma
4) Peripheral T cell lymphomas

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21
Q

What is the “clinical behaviour/natural history” of a disease?

A

Usual course of development of a disease in the absence of treatment

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22
Q

Why do aggressive tumours tend to respond better to chemotherapy?

A

They have higher replicative rate

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23
Q

What are the 4 parameters defining different lymphoma subtypes?

A

1) Clinical features (presentation & clinical course)
2) Morphology
3) Immunophenotype (cell type)
4) Genetics/molecular features

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24
Q

Non-hodgkin lymphomas are (more/less) common than Hodgekin lymphomas.

A

NHL > HL

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25
Q

B cell lymphomas are (more/less) common than T/NK cell lymphomas

A

B cell > T/NK cell lymphomas

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26
Q

Mature lymphomas are (more/less) common than immature/precursor lymphomas

A

Mature > Immature

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27
Q

Classic Hodgkin Lymphoma are (more/less) common than Nodular Lymphocyte Predominant Hodgkin Lymphoma

A

Classic > Nodular

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28
Q

What are the 2 most common B cell lymphomas that, when combined, give rise to >50% of all lymphomas?

A

Diffuse large B cell lymphoma (~31%)

Follicular lymphoma (~22%)

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29
Q

What are 4 main aspects in discerning the clinical features of a lymphoma?

A

1) Nodal vs Extranodal
2) Primary site
3) Immunodeficiency
4) Infection

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30
Q

Which lymphoma is most commonly associated with stomach, thyroid, lung primary site tumours?

A

MALT lymphoma

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31
Q

Which lymphoma(s) is most commonly associated with mediastinum?

A

Adult: Classical HL, Primary mediastinal large B cell lymphoma

Child: Precursor T-lymphoblastic lymphoma

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32
Q

Which lymphoma is most commonly associated with HIV+ px?

A

Burkitt’s lymphoma

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33
Q

Which lymphoma is most commonly associated with transplant px?

A

Post-transplant lymphoproliferative disorders

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34
Q

Which lymphoma(s) is most commonly associated with EBV infection?

A

Burkitt’s lymphoma, Classical HL

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35
Q

Which lymphoma is most commonly associated with H. pylori infection?

A

MALT lymphoma of stomach

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36
Q

What are the 2 main aspects in discerning the morphological features of a lymphoma?

A

1) Growth pattern (diffuse vs nodular)
2) Cell size (large, small, mixed)
3) Characteristic morphology

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37
Q

Which lymphoma is most commonly associated with diffuse growth?

A

Diffuse Large B Cell Lymphoma

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38
Q

Which lymphoma is most commonly associated with nodular growth?

A

Follicular lymphoma

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39
Q

Which lymphoma is most commonly associated with large cells?

A

Diffuse large B cell lymphoma

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40
Q

Which lymphoma(s) is most commonly associated with small cells?

A

Follicular lymphoma
Mantle cell lymphoma

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41
Q

Which lymphoma is most commonly associated with mixed large and small cells?

A

Hodgkin’s lymphoma
T cell lymphoma

42
Q

Which lymphoma is most commonly associated with a Starry Sky appearance?

A

Burkitt’s lymphoma

43
Q

What are the different classifications of surface antigen expression in lymphoma diagnosis and how are they detected?

A

IHC or Flow cytometry

1) Cell origin markers
2) Characteristic markers
3) Specific molecular abnormalities in subtypes

44
Q

Which lymphoma(s) is CD20+?

A

B cell lymphomas

45
Q

Which lymphoma(s) is CD3+?

A

T cell lymphomas

46
Q

Which lymphoma(s) is CD10+ & BCL6+

A

Follicular lymphoma
Burkitt lymphoma

47
Q

Which lymphoma(s) is CD30+ & CD15+?

A

CHL

48
Q

Which lymphoma(s) is BCL2+?

A

Bcl-2 follicular lymphoma

49
Q

Which lymphoma(s) is CCND1+?

A

Mantle cell lymphoma

50
Q

Which lymphoma(s) is Ki67 (proliferation marker) 100%?

A

Burkitt lymphoma

51
Q

Which lymphoma(s) is associated with t(14;18) translocation?

A

Follicular lymphoma

52
Q

Which lymphoma(s) is associated with t(8;14) translocation?

A

Burkitt lymphoma

53
Q

Which lymphoma(s) is associated with t(11;14) translocation?

A

Mantle Cell lymphoma

54
Q

Which lymphoma(s) is associated with t(2;5)?

A

ALK+ Anaplastic Large Cell Lymphoma

55
Q

In what population(s) does DLBCL most commonly occur?

A

Adults and children
- median age 64

56
Q

What is the (i) morphology and (ii) surface Ags in DLBCL?

A

Morphology:
- diffuse
- large cells

Surface Ag:
CD20+ (B cell marker)

57
Q

What is the (i) morphology and (ii) surface Ags (iii) molecular signature of Burkitt’s lymphoma?

A

Morphology:
- Diffuse
- Starry sky (macrophages)

Surface Ag:
- CD20+ (B cell)
- CD10+ (follicle center)

Molecular signature:
- t(8;14)

58
Q

What are the 3 subtypes of Burkitt’s lymphoma?

A

1) Endemic
- African children, EBV+

2) Sporadic
- Western countries children and young adults
- #1 pediatric lymphoma
- ileocecal region

3) Immunodeficient
- HIV+

59
Q

In which population(s) does Burkitt’s lymphoma most commonly occur?

A

Adults and children (median age 31)

EBV+ African Children
HIV+

60
Q

What is the pathogenesis of Burkitt’s lymphoma?

A

t(8;14) → upregulate MYC oncogene (cell cycle regulator)

61
Q

Does follicular lymphoma typically present as a localised tumour or widely disseminated?

A

Widely disseminated
(indolent lymphoma)

62
Q

In which population(s) does Follicular lymphoma most commonly occur?

A

Adults >40 (median: 59)

63
Q

What is the pathogenesis of follicular lymphoma?

A

t(14; 18)(q32, q21)
→ upregulate Bcl2 (anti-apoptotic gene)
→ immortalise lymphoma cells

64
Q

What is the (i) morphology and (ii) surface Ags (iii) molecular signature of follicular lymphoma?

A

Morphology:
- follicular/nodular structure w monotonous accumulation of 1 cell type

Surface Ag:
- CD20+ (B cell)
- CD10+ (follicle center)
- Bcl2

Molecular signature:
t(14; 18)(q32, q21) detected by (i) cytogenetics (ii) FISH or (iii) PCR

65
Q

What is a classical Hodgkin Lymphoma?

A

Lymphoid neoplasm composed of Reed-sternberg (neoplastic) cells in a inflammatory (non-neoplastic) background. (lymphocytes, histiocytes, eosinophils, plasma cells, etc.)

66
Q

In which population(s) does Classic Hodgkin lymphoma most commonly occur?

A

Late adolescents/young adults and 60s (bimodal curve shifts to younger ages in poorer countries)

67
Q

How is CHL diagnosed?

A

Histological findings:
1) Background rich in inflammatory cells, esp eosinophils
2) Presence of Reed-Sternberg (lacunar) cells

68
Q

How does Hodgkin lymphoma spread?

A

Contiguously via lymphatics (cannot be diffuse)

69
Q

How is CHL treated?

A

Limited stage:
- radiation therapy

Higher stage:
- multi-agent chemotherapy ±radiation therapy

70
Q

What is the general prognosis for px with CHL?

A

Curable (~80%)

71
Q

What cell lineage is CHL from?

A

B cell

72
Q

What are the Ix for a suspected lymphoma?

A

1) FNAC (fine needle aspiration cytology)
2) Biopsy
- Flow cytometry
- Histology
- IHC
- Cytogenetics
- Molecular tests

73
Q

Which organs do primary neoplasms most commonly metastasise to?

A

Lymph nodes

74
Q

How do carcinomas usually spread?

A

By lymphatics

75
Q

How do sarcomas usually spread?

A

Hematogenously

76
Q

How can one identify the source of metastatic tumour?

A

1) Microscopic features
2) Location of Lymph node involvement
3) IHC
4) Imaging (CT/PET)

77
Q

What are the differences between lymphomas and leukemias?

A

1) Bone marrow vs LN and extra nodal
2) Widespread involvement of bone marrow vs discrete tumour masses
3) Large no. tumour cells in peripheral blood in leukemias

78
Q

What are the main cells present in normal bone marrow on histology?

A

1) Erythroid series (erythrocytes)
2) Myeloid series (polymorph)
3) Megakaryocytic series
4) Others (lymphocytes, plasma cells)

79
Q

What is the typical histological appearance of bone marrow in leukemia?

A

1) Hypercellularity (>90%)
2) Monotony (1 predominant neoplastic cell type)

80
Q

What are the differences between acute and chronic leukemias?

A

1) Prognosis if untreated (acute: fatal)
2) Symptoms (acute: BM suppression, chronic: non-specific)
3) Histology
(acute: immature blast cells, chronic: mature well-differentiated cells)

81
Q

What are the 4 general types of leukemias?

A

1) Acute Lymphoblastic (ALL)
2) Chronic Lymphoid (CLL)
3) Acute Myeloblastic (AML)
4) Chronic myeloid (CML)

82
Q

How are leukemias diagnosed?

A

1) Clinical
- abnormal blood count
- symptoms and signs
- sites of involvement

2) Morphological
- degree of differentiation

3) Surface Ags

4) Molecular/genetic abnormalities

83
Q

What is the pathology of CML?

A

t(9;22)
→ BCR:ABL1
→ constitutively active tyrosine kinase
(can use TKIs)

84
Q

What are the 2 functional categories of tissue in the spleen?

A

White pulp (lymphoreticular)
- arteries
- T/B cells

Red pulp (vascular)
- venous sinusoids
- macrophages

85
Q

What is the key symptoms in spleen diseases?

A

Splenomegaly

86
Q

What are 5 causes of splenomegaly?

A

1) Infections (any blood borne)
2) Congestion (portal hypertension)
3) Lympho-hematogenous disorders (lymphomas, leukemias, anemias)
4) Immunologic-inflammatory conditions (infarcts, atheromatous disease)
5) Storage diseases
6) Others

87
Q

What are the the 2 structural components of the thymus?

A

1) Lymphoid component
2) Epithelial component (Hassall corpuscles)

88
Q

What are some causes of thymic disease?

A

1) Developmental disorders
2) Thymic hyperplasias
3) Myasthenia gravis
4) Thymic (mediastinal) neoplasms

89
Q

True or false: Thymomas are
much more common in adults than in children

A

True

90
Q

True or false: NHL and HL are much more common in children than adults

A

True

91
Q

Which organ is enlarged in portal hypertension?

A

Spleen

92
Q

How do you differentiate a normal and diseased spleen on gross anatomy?

A

Disease spleen has obtuse rounded edges (top and bottom)

93
Q

What are the gross morphological features of a splenic infarct?

A

Sharply defined (vascular cause) white infarct
- surrounded by redness (hyperaemia from inflammation)

94
Q

What are the gross morphological features of miliary TB?

A

Multiple areas of caseous necrosis
(smaller area=newer)

95
Q

How is miliary TB spread?

A

Hematogenously

96
Q

What are the gross morphological features of Hodgkin lymphoma?

A

Enlarged spleen (+obtuse angles) with multiple small white nodules

97
Q

What are the gross morphological features of Spleen lymphoma?

A

Enlarged spleen with ill-defined/diffuse areas of pallor (from internal necrosis)

98
Q

What are the gross morphological features of Spleen leukemia?

A

Enlarged spleen with dusky appearance

99
Q

What are the histological features of non-Hodgkin lymphoma?

A

1) Diffuse effacement of lymph node architecture (no follicles)
2) Infiltration by abnormal large lymphoid cells with large/multiple pleomorphic nuclei/nucleoli
3) Monotonous sheets of 1 predominant cell type

100
Q

What are the histological features of a metastatic squamous cell carcinoma?

A

1) Necrosis
2) Cohesive squamous epithelial cells with intercellular bridges ( “Whorled” appearance)
3) Keratin pearls
4) Dense cytoplasm