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
B cell lymphomas are (more/less) common than T/NK cell lymphomas
B cell > T/NK cell lymphomas
26
Mature lymphomas are (more/less) common than immature/precursor lymphomas
Mature > Immature
27
Classic Hodgkin Lymphoma are (more/less) common than Nodular Lymphocyte Predominant Hodgkin Lymphoma
Classic > Nodular
28
What are the 2 most common B cell lymphomas that, when combined, give rise to >50% of all lymphomas?
Diffuse large B cell lymphoma (~31%) Follicular lymphoma (~22%)
29
What are 4 main aspects in discerning the clinical features of a lymphoma?
1) Nodal vs Extranodal 2) Primary site 3) Immunodeficiency 4) Infection
30
Which lymphoma is most commonly associated with stomach, thyroid, lung primary site tumours?
MALT lymphoma
31
Which lymphoma(s) is most commonly associated with mediastinum?
Adult: Classical HL, Primary mediastinal large B cell lymphoma Child: Precursor T-lymphoblastic lymphoma
32
Which lymphoma is most commonly associated with HIV+ px?
Burkitt's lymphoma
33
Which lymphoma is most commonly associated with transplant px?
Post-transplant lymphoproliferative disorders
34
Which lymphoma(s) is most commonly associated with EBV infection?
Burkitt's lymphoma, Classical HL
35
Which lymphoma is most commonly associated with H. pylori infection?
MALT lymphoma of stomach
36
What are the 2 main aspects in discerning the morphological features of a lymphoma?
1) Growth pattern (diffuse vs nodular) 2) Cell size (large, small, mixed) 3) Characteristic morphology
37
Which lymphoma is most commonly associated with diffuse growth?
Diffuse Large B Cell Lymphoma
38
Which lymphoma is most commonly associated with nodular growth?
Follicular lymphoma
39
Which lymphoma is most commonly associated with large cells?
Diffuse large B cell lymphoma
40
Which lymphoma(s) is most commonly associated with small cells?
Follicular lymphoma Mantle cell lymphoma
41
Which lymphoma is most commonly associated with mixed large and small cells?
Hodgkin's lymphoma T cell lymphoma
42
Which lymphoma is most commonly associated with a Starry Sky appearance?
Burkitt's lymphoma
43
What are the different classifications of surface antigen expression in lymphoma diagnosis and how are they detected?
IHC or Flow cytometry 1) Cell origin markers 2) Characteristic markers 3) Specific molecular abnormalities in subtypes
44
Which lymphoma(s) is CD20+?
B cell lymphomas
45
Which lymphoma(s) is CD3+?
T cell lymphomas
46
Which lymphoma(s) is CD10+ & BCL6+
Follicular lymphoma Burkitt lymphoma
47
Which lymphoma(s) is CD30+ & CD15+?
CHL
48
Which lymphoma(s) is BCL2+?
Bcl-2 follicular lymphoma
49
Which lymphoma(s) is CCND1+?
Mantle cell lymphoma
50
Which lymphoma(s) is Ki67 (proliferation marker) 100%?
Burkitt lymphoma
51
Which lymphoma(s) is associated with t(14;18) translocation?
Follicular lymphoma
52
Which lymphoma(s) is associated with t(8;14) translocation?
Burkitt lymphoma
53
Which lymphoma(s) is associated with t(11;14) translocation?
Mantle Cell lymphoma
54
Which lymphoma(s) is associated with t(2;5)?
ALK+ Anaplastic Large Cell Lymphoma
55
In what population(s) does DLBCL most commonly occur?
Adults and children - median age 64
56
What is the (i) morphology and (ii) surface Ags in DLBCL?
Morphology: - diffuse - large cells Surface Ag: CD20+ (B cell marker)
57
What is the (i) morphology and (ii) surface Ags (iii) molecular signature of Burkitt's lymphoma?
Morphology: - Diffuse - Starry sky (macrophages) Surface Ag: - CD20+ (B cell) - CD10+ (follicle center) Molecular signature: - t(8;14)
58
What are the 3 subtypes of Burkitt's lymphoma?
1) Endemic - African children, EBV+ 2) Sporadic - Western countries children and young adults - #1 pediatric lymphoma - ileocecal region 3) Immunodeficient - HIV+
59
In which population(s) does Burkitt's lymphoma most commonly occur?
Adults and children (median age 31) EBV+ African Children HIV+
60
What is the pathogenesis of Burkitt's lymphoma?
t(8;14) → upregulate MYC oncogene (cell cycle regulator)
61
Does follicular lymphoma typically present as a localised tumour or widely disseminated?
Widely disseminated (indolent lymphoma)
62
In which population(s) does Follicular lymphoma most commonly occur?
Adults >40 (median: 59)
63
What is the pathogenesis of follicular lymphoma?
t(14; 18)(q32, q21) → upregulate Bcl2 (anti-apoptotic gene) → immortalise lymphoma cells
64
What is the (i) morphology and (ii) surface Ags (iii) molecular signature of follicular lymphoma?
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
What is a classical Hodgkin Lymphoma?
Lymphoid neoplasm composed of Reed-sternberg (neoplastic) cells in a inflammatory (non-neoplastic) background. (lymphocytes, histiocytes, eosinophils, plasma cells, etc.)
66
In which population(s) does Classic Hodgkin lymphoma most commonly occur?
Late adolescents/young adults and 60s (bimodal curve shifts to younger ages in poorer countries)
67
How is CHL diagnosed?
Histological findings: 1) Background rich in inflammatory cells, esp eosinophils 2) Presence of Reed-Sternberg (lacunar) cells
68
How does Hodgkin lymphoma spread?
Contiguously via lymphatics (cannot be diffuse)
69
How is CHL treated?
Limited stage: - radiation therapy Higher stage: - multi-agent chemotherapy ±radiation therapy
70
What is the general prognosis for px with CHL?
Curable (~80%)
71
What cell lineage is CHL from?
B cell
72
What are the Ix for a suspected lymphoma?
1) FNAC (fine needle aspiration cytology) 2) Biopsy - Flow cytometry - Histology - IHC - Cytogenetics - Molecular tests
73
Which organs do primary neoplasms most commonly metastasise to?
Lymph nodes
74
How do carcinomas usually spread?
By lymphatics
75
How do sarcomas usually spread?
Hematogenously
76
How can one identify the source of metastatic tumour?
1) Microscopic features 2) Location of Lymph node involvement 3) IHC 4) Imaging (CT/PET)
77
What are the differences between lymphomas and leukemias?
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
What are the main cells present in normal bone marrow on histology?
1) Erythroid series (erythrocytes) 2) Myeloid series (polymorph) 3) Megakaryocytic series 4) Others (lymphocytes, plasma cells)
79
What is the typical histological appearance of bone marrow in leukemia?
1) Hypercellularity (>90%) 2) Monotony (1 predominant neoplastic cell type)
80
What are the differences between acute and chronic leukemias?
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
What are the 4 general types of leukemias?
1) Acute Lymphoblastic (ALL) 2) Chronic Lymphoid (CLL) 3) Acute Myeloblastic (AML) 4) Chronic myeloid (CML)
82
How are leukemias diagnosed?
1) Clinical - abnormal blood count - symptoms and signs - sites of involvement 2) Morphological - degree of differentiation 3) Surface Ags 4) Molecular/genetic abnormalities
83
What is the pathology of CML?
t(9;22) → BCR:ABL1 → constitutively active tyrosine kinase (can use TKIs)
84
What are the 2 functional categories of tissue in the spleen?
White pulp (lymphoreticular) - arteries - T/B cells Red pulp (vascular) - venous sinusoids - macrophages
85
What is the key symptoms in spleen diseases?
Splenomegaly
86
What are 5 causes of splenomegaly?
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
What are the the 2 structural components of the thymus?
1) Lymphoid component 2) Epithelial component (Hassall corpuscles)
88
What are some causes of thymic disease?
1) Developmental disorders 2) Thymic hyperplasias 3) Myasthenia gravis 4) Thymic (mediastinal) neoplasms
89
True or false: Thymomas are much more common in adults than in children
True
90
True or false: NHL and HL are much more common in children than adults
True
91
Which organ is enlarged in portal hypertension?
Spleen
92
How do you differentiate a normal and diseased spleen on gross anatomy?
Disease spleen has obtuse rounded edges (top and bottom)
93
What are the gross morphological features of a splenic infarct?
Sharply defined (vascular cause) white infarct - surrounded by redness (hyperaemia from inflammation)
94
What are the gross morphological features of miliary TB?
Multiple areas of caseous necrosis (smaller area=newer)
95
How is miliary TB spread?
Hematogenously
96
What are the gross morphological features of Hodgkin lymphoma?
Enlarged spleen (+obtuse angles) with multiple small white nodules
97
What are the gross morphological features of Spleen lymphoma?
Enlarged spleen with ill-defined/diffuse areas of pallor (from internal necrosis)
98
What are the gross morphological features of Spleen leukemia?
Enlarged spleen with dusky appearance
99
What are the histological features of non-Hodgkin lymphoma?
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
What are the histological features of a metastatic squamous cell carcinoma?
1) Necrosis 2) Cohesive squamous epithelial cells with intercellular bridges ( "Whorled" appearance) 3) Keratin pearls 4) Dense cytoplasm