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Haematology and Immunology > Lymphoid Malignancy > Flashcards

Lymphoid Malignancy Flashcards

1
Q

What is leukaemia cancers of?

A

Lymphoid origin

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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
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3
Q

What test tells us the type of leukaemia?

A

Biopsy (lymph node, bone marrow etc)

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4
Q

What test tells us where the leukaemia is?

A

Clinical exam

imaging (e.g. CT)

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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)

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6
Q

Lymphoproliferative disorders

A

Acute lymphoblastic leukaemia (ALL)
Chronic lymphocytic leukaemia (CLL)
Hodgkin Lymphoma
Non-Hodgkin Lymphoma (NHL)

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7
Q

Types of Non-Hodgkin Lymphoma (NHL)

A

High grade
- diffuse large B cell lymphoma
Low grade
- follicular, marginal zone

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8
Q

What is acute lymphoblastic leukaemia (ALL)?

A

Neoplastic disorder of lymphoblasts

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9
Q

How is ALL diagnosed?

A

By > 20% lymphoblasts present in bone marrow

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10
Q

Who does 75% of cases of ALL occur in?

A

Children < 6 y/o

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11
Q

How much of ALL is B cell lineage?

A

75-90%

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12
Q

Presentation of ALL

A

2-3 week history of bone marrow failure +/- raised white cell count or bone/joint pain
Infection
Sweats

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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
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14
Q

S/Es of T cell immunotherapy

A

Cytokine release syndrome

Neurotoxicity

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15
Q

Presentation of cytokine release syndrome

A

Fever
Hypotension
Dyspnoea

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16
Q

Presentation of neurotoxicity

A 
Confusion with normal conscious level 
seizure
headache
focal neurology
coma
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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
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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%
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19
Q

What is the commonest leukaemia worldwide?

A

Chronic Lymphocytic Leukaemia (CLL)

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20
Q

Which gender gets CLL?

A

M > F 2:1

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21
Q

Where is CLL rare?

A

Far east

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22
Q

Presentation of CLL

A 
Often asymptomatic at presentation 
Bone marrow failure
- anaemia
- thrombocytopenia 
Lymphadenopathy 
Splenomegaly (30%)
Fever and sweats (<25%)
Hepatomegaly
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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
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24
Q

Associated findings of CLL

A 
Immune Paresis (loss of normal immunoglobulin production) 
Haemolytic anaemia
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25
Q

Binet staging of CLL

A 
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
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26
Q

Indications for treatment of CLL

A 
Progressive bone marrow failure 
Massive lymphadenopathy 
Progressive splenomegaly 
Lymphocyte doubling time < 6 months or > 50% increase over 2 months
Systemic symptoms 
Autoimmune cytopenias
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27
Q

Treatment of CLL

A 
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
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28
Q

Poor prognostic markers of CLL

A

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

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29
Q

Presentation of lymphoma

A

Lymphadenopathy / Hepatosplenomegaly
Extranodal disease
B symptoms
Bone marrow involvement

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30
Q

Assessment of staging of lymphoma

A

Lymph node biopsy
CT scan
Bone marrow aspirate
Trephine

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31
Q

Non-Hodgkin Lymphoma is classified according to what?

A 
Lineage - B or T cell 
Grade of disease 
- high grade
- low grade
Histological features of disease
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32
Q

What is the majority of lineage of Non-Hodgkin’s lymphoma?

A

B cell in origin

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33
Q

Features of high grade Non-Hodgkin Lymphoma

A

Aggressive
fast growing
requires combination chemotherapy
Can be cured, but again varies widely

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34
Q

Features of low grade Non-Hodgkin lymphoma

A

Indolent, often asymptomatic
Responds to chemo but incurable
Medial survival varies by subtype

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35
Q

Specific disease entities of Non-Hodgkin Lymphoma

A

Diffuse large B cell lymphoma

Follicular lymphoma

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36
Q

What is the commonest subtype of lymphoma?

A

Diffuse large B cell lymphoma

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37
Q

Is diffuse large B cell non-hodgkins lymphoma high grade or low grade?

A

High grade

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38
Q

Is follicular lymphoma a high grade or low grade lymphoma?

A

Low grade

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39
Q

Who gets Hodgkin Lymphoma?

A

1st peak 15 - 35 y/o
2nd peak later in life
M > F 1.9:1

40
Q

Associations of Hodgkin lymphoma

A

Epstein Barr virus
Familial
Geographical clustering

41
Q

Treatment of Hodgkin Lymphoma

A 
Combination chemotherapy (ABVD)
\+ / - radiotherapy
Monoclonal antibodies (anti-CD30)
Immunotherapy (checkpoint inhibitors)
42
Q

What is used in Hodgkin Lymphoma to assess response to treatment and limit use of radiotherapy?

A

PET scan

43
Q

What does the bone marrow produce?

A

Granulocytes
Red cells
Platelets
Precursor lymphocytes

44
Q

The granulocytes, red cells and platelets are released into where? Are they precursor cells or ready to work?

A

Blood

Ready to work

45
Q

What kind of immune system are the precursor lymphocytes involved in?

A

Adaptive immune system

46
Q

What do B precursor lymphocytes become?

A

Plasma cells that produce specific antibodies

47
Q

Where are B precursor lymphocytes found when they are maturing?

A

The pale bit of a lymph node

48
Q

What cells are over proliferating in AML?

A

Myeloid progenitor cells / myeloblast

49
Q

What do myeloproliferative disorders involve?

A

Mutations after differentiation but still uncontrolled proliferation

50
Q

What cells are over proliferating in ALL?

A

Lymphoid progenitor cells / lymphoblasts

51
Q

What are the lymph organs?

A 
Lymph nodes
Spleen 
Tonsils
Adenoids
MALT
52
Q

What structures are involved in MALT?

A

Gut
Bronchus
Salivary glands

53
Q

Where does the mutation occur to give lymphoma?

A

Post bone marrow

54
Q

When does leukaemia occur?

A

When bone marrow spills into the blood

55
Q

What blood changes are likely to occur in bacterial meningitis?

A

Increase in neutrophils

56
Q

What blood changes are likely to be seen in leukaemia?

A

Blasts

Pancytopenia

57
Q

What is the bleeding time?

A

Formation of a platelet clot

58
Q

What is the clotting time?

A

Formation of a thrombin clot

59
Q

What is the normal lymphoblast count?

A

0

60
Q

What test would differentiate between AML and ALL?

A

Flow cytometry

61
Q

What would be found in the differences between AML and ALL?

A

Different antigens on the lymphoblasts

62
Q

Why do blood cells flow well on flow cytometry?

A

They are single cells

63
Q

What does cytogenetics tell us?

A

If there are poor prognostic markers

64
Q

What results in cytogenetics indicate high risk groups for ALL?

A

t (9;22)

t (4;11)

65
Q

What is the classification of weight loss as a B symptom?

A

> 10% weight loss in 6 months

66
Q

Lymphoblasts vs lymphocytes on blood film?

A

Lymphoblasts much bigger on blood film

67
Q

What type of cells are classic on a blood film of CLL?

A

Smudge cells

68
Q

What type of cells are involved in CLL?

A

Naïve mature lymphocytes

69
Q

What can CLL present as? Why?

A

Lymphoma or Leukaemia

Can have lymphocytosis in blood and in bone marrow OR can be just in lymph nodes

70
Q

As soon as there is lymphocytosis in the blood, what is the condition?

A

Leukaemia

71
Q

Lymphocytosis just in the lymph node is known as what?

A

Small lymphocytic lymphoma

72
Q

When to treat CLL?

A 
Uncontrolled cytopenias
Autoimmune haemolytic complications
B symptoms
Bulky lymph nodes
Splenomegaly
73
Q

What is another name for DAT?

A

Coombs test

74
Q

What is a cyst on the side of the neck called?

A

Brachial cyst

75
Q

What is a cyst on the midline of the neck called?

A

Thyroglossal cyst

76
Q

Which lymphomas are common in the young?

A

Hodgkins

Burkitts

77
Q

What is Hodgkins lymphoma defined by?

A

A cell in the lymph node

78
Q

Why can Hodgkins lymphoma have a bacterial look about it?

A

As cytokines are produced - making you think it may be bacterial

79
Q

What do lymphomas often have (to do with blood)?

A

Anaemia

80
Q

If miss the cell in a hodgkins node, what is the next test?

A

An excision biopsy

81
Q

Key diagnostic test for HL

A

Nodal biopsy

82
Q

When would a PET scan light up, not related to malignancy?

A

Site of surgery

83
Q

Complication of burkitts lymphoma which has been treated with chemotherapy

A

Tumour lysis syndrome

84
Q

Hodgkins lymphoma can cause severe pain when doing what? How many people does this occur in?

A

Drinking alcohol

In 10% of patients

85
Q

What type of cells are present in HL?

A

Reed-Sternberg cells

86
Q

What can CLL turn into, what is this called and what is the presentation?

A 
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
Q

Type of HL with worst prognosis

A

Lymphocyte depleted

88
Q

Type of HL with best prognosis

A

Lymphocyte predominant

89
Q

What may myelodysplasia progress to?

A

AML

90
Q

Most common histological type of HL

A

Nodular sclerosing

91
Q

Poor prognosis factors for HL

A 
Age > 45
Male
Stage IV disease
Haemoglobin < 10.5g/L
Lymphocyte count < 600/ul or < 8%
Albumin < 40 
WBC > 15,000
92
Q

What does Burkitts lymphoma look like on lymph node biopsy?

A

‘Starry sky’ appearance

93
Q

What is Burkitts lymphoma associated with?

A

EBV

94
Q

What malignancy may present with asymmetrical spreading lymphadenopathy?

A

HL

95
Q

What gene translocation is related to Burkitts lymphoma?

A

C-myc translocation

96
Q

Complication of CLL

A

Recurrent infection (due to hypogammaglobulinaemia)

Haematology and Immunology (18 decks)

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