Lymphoid Malignancy Flashcards
1
Q
What is leukaemia cancers of?
A
Lymphoid origin
2
Q
Presentation of leukaemia
A
Enlarged lymph nodes (lymphadenopathy) OR with extranodal involvement OR with bone marrow involvement Systemic symptoms - weight loss (>10% in 6 months) - fever - night sweats - pruritis - fatigue
3
Q
What test tells us the type of leukaemia?
A
Biopsy (lymph node, bone marrow etc)
4
Q
What test tells us where the leukaemia is?
A
Clinical exam
imaging (e.g. CT)
5
Q
What is a broad difference of Hodgkin Lymphoma vs Non-Hodgkin Lymphoma?
A
Hodgkin = specific disease
Non - Hodgkin = everything else (approx. 50 subtypes)
6
Q
Lymphoproliferative disorders
A
Acute lymphoblastic leukaemia (ALL)
Chronic lymphocytic leukaemia (CLL)
Hodgkin Lymphoma
Non-Hodgkin Lymphoma (NHL)
7
Q
Types of Non-Hodgkin Lymphoma (NHL)
A
High grade
- diffuse large B cell lymphoma
Low grade
- follicular, marginal zone
8
Q
What is acute lymphoblastic leukaemia (ALL)?
A
Neoplastic disorder of lymphoblasts
9
Q
How is ALL diagnosed?
A
By > 20% lymphoblasts present in bone marrow
10
Q
Who does 75% of cases of ALL occur in?
A
Children < 6 y/o
11
Q
How much of ALL is B cell lineage?
A
75-90%
12
Q
Presentation of ALL
A
2-3 week history of bone marrow failure +/- raised white cell count or bone/joint pain
Infection
Sweats
13
Q
Treatment of ALL
A
Induction chemo to obtain remission Consolidation therapy CNS directed treatment Maintenance therapy for 18 months Allogenic stem cell transplantation (if HIGH RISK) newer therapies - Bispecific T cell engagers - CAR
14
Q
S/Es of T cell immunotherapy
A
Cytokine release syndrome
Neurotoxicity
15
Q
Presentation of cytokine release syndrome
A
Fever
Hypotension
Dyspnoea
16
Q
Presentation of neurotoxicity
A
Confusion with normal conscious level seizure headache focal neurology coma
17
Q
Poor risk factors for ALL
A
Increasing age increased WCC immunophenotyped (more primitive forms) Cytogenetics/molecular genetics (t(9;22), t(4;11)) Slow/poor response to treatment
18
Q
Prognosis of ALL
A
Adults - complete remission 78-91% - leukaemia free survival a 5 y - 30-35% Children - 5 yr overall survival 90% - Poor risk patients (slow response to induction or Philadelphia positive) 5 yr overall survival 45%
19
Q
What is the commonest leukaemia worldwide?
A
Chronic Lymphocytic Leukaemia (CLL)
20
Q
Which gender gets CLL?
A
M > F 2:1
21
Q
Where is CLL rare?
A
Far east
22
Q
Presentation of CLL
A
Often asymptomatic at presentation Bone marrow failure - anaemia - thrombocytopenia Lymphadenopathy Splenomegaly (30%) Fever and sweats (<25%) Hepatomegaly
23
Q
Diagnosis of CLL
A
Blood > 5 x10 9/L lymphocytes Bone marrow > 30% lymphocytes Characteristic immunophenotyping - B cell markers (CD 19, 20, 23) - CD5 positive
24
Q
Associated findings of CLL
A
Immune Paresis (loss of normal immunoglobulin production) Haemolytic anaemia
25
Binet staging of CLL
```
Stage A
- < 3 lymph node areas
- same survival as matched controls
Stage B
- 3 or more lymph node areas
- survival approx. 8 years
Stage C
- stage B and anaemia or thrombocytopenia
- survival approx. 6 years
```
26
Indications for treatment of CLL
```
Progressive bone marrow failure
Massive lymphadenopathy
Progressive splenomegaly
Lymphocyte doubling time < 6 months or > 50% increase over 2 months
Systemic symptoms
Autoimmune cytopenias
```
27
Treatment of CLL
```
Often nothing - watch and wait
Cytotoxic chemotherapy e.g. fludarabine
Monoclonal antibodies e.g. rituximab
Novel agents
- bruton tyrokinase inhibitor
- PI3K inhibitor
- BCL-2 inhibitor
```
28
Poor prognostic markers of CLL
Advanced disease (Binet Stage B or C)
Atypical lymphocytes morphology
Rapid lymphocyte doubling time < 12 months
CD38 + expression
Loss / mutation p53; dell 11q23 (ATM gene)
Unmutated IgVH gene status
29
Presentation of lymphoma
Lymphadenopathy / Hepatosplenomegaly
Extranodal disease
B symptoms
Bone marrow involvement
30
Assessment of staging of lymphoma
Lymph node biopsy
CT scan
Bone marrow aspirate
Trephine
31
Non-Hodgkin Lymphoma is classified according to what?
```
Lineage - B or T cell
Grade of disease
- high grade
- low grade
Histological features of disease
```
32
What is the majority of lineage of Non-Hodgkin's lymphoma?
B cell in origin
33
Features of high grade Non-Hodgkin Lymphoma
Aggressive
fast growing
requires combination chemotherapy
Can be cured, but again varies widely
34
Features of low grade Non-Hodgkin lymphoma
Indolent, often asymptomatic
Responds to chemo but incurable
Medial survival varies by subtype
35
Specific disease entities of Non-Hodgkin Lymphoma
Diffuse large B cell lymphoma
| Follicular lymphoma
36
What is the commonest subtype of lymphoma?
Diffuse large B cell lymphoma
37
Is diffuse large B cell non-hodgkins lymphoma high grade or low grade?
High grade
38
Is follicular lymphoma a high grade or low grade lymphoma?
Low grade
39
Who gets Hodgkin Lymphoma?
1st peak 15 - 35 y/o
2nd peak later in life
M > F 1.9:1
40
Associations of Hodgkin lymphoma
Epstein Barr virus
Familial
Geographical clustering
41
Treatment of Hodgkin Lymphoma
```
Combination chemotherapy (ABVD)
+ / - radiotherapy
Monoclonal antibodies (anti-CD30)
Immunotherapy (checkpoint inhibitors)
```
42
What is used in Hodgkin Lymphoma to assess response to treatment and limit use of radiotherapy?
PET scan
43
What does the bone marrow produce?
Granulocytes
Red cells
Platelets
Precursor lymphocytes
44
The granulocytes, red cells and platelets are released into where? Are they precursor cells or ready to work?
Blood
| Ready to work
45
What kind of immune system are the precursor lymphocytes involved in?
Adaptive immune system
46
What do B precursor lymphocytes become?
Plasma cells that produce specific antibodies
47
Where are B precursor lymphocytes found when they are maturing?
The pale bit of a lymph node
48
What cells are over proliferating in AML?
Myeloid progenitor cells / myeloblast
49
What do myeloproliferative disorders involve?
Mutations after differentiation but still uncontrolled proliferation
50
What cells are over proliferating in ALL?
Lymphoid progenitor cells / lymphoblasts
51
What are the lymph organs?
```
Lymph nodes
Spleen
Tonsils
Adenoids
MALT
```
52
What structures are involved in MALT?
Gut
Bronchus
Salivary glands
53
Where does the mutation occur to give lymphoma?
Post bone marrow
54
When does leukaemia occur?
When bone marrow spills into the blood
55
What blood changes are likely to occur in bacterial meningitis?
Increase in neutrophils
56
What blood changes are likely to be seen in leukaemia?
Blasts
| Pancytopenia
57
What is the bleeding time?
Formation of a platelet clot
58
What is the clotting time?
Formation of a thrombin clot
59
What is the normal lymphoblast count?
0
60
What test would differentiate between AML and ALL?
Flow cytometry
61
What would be found in the differences between AML and ALL?
Different antigens on the lymphoblasts
62
Why do blood cells flow well on flow cytometry?
They are single cells
63
What does cytogenetics tell us?
If there are poor prognostic markers
64
What results in cytogenetics indicate high risk groups for ALL?
t (9;22)
| t (4;11)
65
What is the classification of weight loss as a B symptom?
> 10% weight loss in 6 months
66
Lymphoblasts vs lymphocytes on blood film?
Lymphoblasts much bigger on blood film
67
What type of cells are classic on a blood film of CLL?
Smudge cells
68
What type of cells are involved in CLL?
Naïve mature lymphocytes
69
What can CLL present as? Why?
Lymphoma or Leukaemia
| Can have lymphocytosis in blood and in bone marrow OR can be just in lymph nodes
70
As soon as there is lymphocytosis in the blood, what is the condition?
Leukaemia
71
Lymphocytosis just in the lymph node is known as what?
Small lymphocytic lymphoma
72
When to treat CLL?
```
Uncontrolled cytopenias
Autoimmune haemolytic complications
B symptoms
Bulky lymph nodes
Splenomegaly
```
73
What is another name for DAT?
Coombs test
74
What is a cyst on the side of the neck called?
Brachial cyst
75
What is a cyst on the midline of the neck called?
Thyroglossal cyst
76
Which lymphomas are common in the young?
Hodgkins
| Burkitts
77
What is Hodgkins lymphoma defined by?
A cell in the lymph node
78
Why can Hodgkins lymphoma have a bacterial look about it?
As cytokines are produced - making you think it may be bacterial
79
What do lymphomas often have (to do with blood)?
Anaemia
80
If miss the cell in a hodgkins node, what is the next test?
An excision biopsy
81
Key diagnostic test for HL
Nodal biopsy
82
When would a PET scan light up, not related to malignancy?
Site of surgery
83
Complication of burkitts lymphoma which has been treated with chemotherapy
Tumour lysis syndrome
84
Hodgkins lymphoma can cause severe pain when doing what? How many people does this occur in?
Drinking alcohol
| In 10% of patients
85
What type of cells are present in HL?
Reed-Sternberg cells
86
What can CLL turn into, what is this called and what is the presentation?
```
High grade lymphoma (fast growing diffuse large B cell non hodgkins lymphoma)
Called Richters transformation
Presentation
- patient becomes unwell VERY suddenly
- lymph node swelling
- fever without infection
- weight loss
- night sweats
- nausea
- abdominal pain
```
87
Type of HL with worst prognosis
Lymphocyte depleted
88
Type of HL with best prognosis
Lymphocyte predominant
89
What may myelodysplasia progress to?
AML
90
Most common histological type of HL
Nodular sclerosing
91
Poor prognosis factors for HL
```
Age > 45
Male
Stage IV disease
Haemoglobin < 10.5g/L
Lymphocyte count < 600/ul or < 8%
Albumin < 40
WBC > 15,000
```
92
What does Burkitts lymphoma look like on lymph node biopsy?
'Starry sky' appearance
93
What is Burkitts lymphoma associated with?
EBV
94
What malignancy may present with asymmetrical spreading lymphadenopathy?
HL
95
What gene translocation is related to Burkitts lymphoma?
C-myc translocation
96
Complication of CLL
Recurrent infection (due to hypogammaglobulinaemia)
