Lymphoid Malignancies Flashcards

1
Q

Define lymphoma

A

Cancer of lymphoid origin

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2
Q

Presentation of lymphoma

A

Node enlargement
Extranodal disease
Bone Marrow Involvement
Systemic B Symptoms

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3
Q

Name the Systemic ‘B’ Symptoms

A
Fever
Weight loss (>10% within 6 months, unintentional)
Drenching sweats
Pruritus
Fatigue
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4
Q

How do you diagnose lymphoma?

A

Malignant cell characteristics seen on Biopsy

Clinical Exam and Imaging

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5
Q

How do you stage lymphoma and for what reason?

A

Clinical exam and imaging
To locate and determine extent of disease
Prognosis
Treatment

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6
Q

Name 4 lymhoproliferative disorders

A

Acute Lymphoblastic leukaemia
Chronic Lymphocytic leukaemia
Hodgkin’s lymphoma
Non-hodgkin’s lymphoma

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7
Q

Most common lymphoproliferative disorder in clinical practice

A

NHL

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8
Q

2nd most common lymphoproliferative disorder in clinical practice

A

Chronic Lymphocytic Leukaemia

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9
Q

In which condition do 75% of cases occur in children under 6?

A

Acute lymphoblastic Leukaemia

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10
Q

Diagnosis- presents with 2/3 week history of bone marrow failure, bone/joint pain?

A

Acute lymphoblastic leukaemia

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11
Q

Child presents with limp- blood count normal?

A

Acute lymphoblastic leukaemia

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12
Q

Investigations for ALL

A

Blood count
Blood film
Bone marrow sample

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13
Q

Features of blood result for ALL

A

Haemoglobin low
White blood cells high
Platelets low

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14
Q

What cell type constitutes 90% of a bone marrow sample taken from patient with ALL?

A

B lymphocytes

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15
Q

What markers will be present in cells in an ALL sample?

A

CD19 - present on all B cells

CD34 & TDT - markers of v early immature cells - no differentiation

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16
Q

2 treatment options for ALL?

A

Standard treatment - chemotherapy

Stem cell transplantation (if very high risk)

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17
Q

Describe the standard treatment of ALL?

A

Induction chemotherapy for remission
Consolidation therapy
CNS directed treatment
Maintenance chemotherapy for 18 months

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18
Q

Name 2 new T cell therapies

A

BiTE - BiSpecific T cell Engager - blinatumomab

CAR T cells

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19
Q

Mechanism of BiTe treatment

A

‘Supercharge’ T cells

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20
Q

Mechanism of CAR T cells

A

Harvest and modify T cells, reinfuse in patient

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21
Q

Side effects of T cell immunotherapy

A

Cytokine release syndrome

Neurotoxicity

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22
Q

Features of cytokine release syndrome

A

Fever
Hypotension
Dyspnoea

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23
Q

Signs of neurotoxicity

A
Confusion but normal consciousness level
Seizures
Focal neurology
Coma
Headaches
24
Q

Cytokine Release Syndrome is a side effect most commonly associated with which therapy

A

CAR T cell therapy (Chimeric Antigen Receptor)

25
Poor Risk factors for ALL
Increased Age Increased WBC Genetics BCR-ABL mutation t(9; 22) Slow/poor response to treatment
26
Remission rate of adults with ALL
90%
27
5 year survival in children with ALL
90%
28
5 Year overall survival in at risk patients with ALL
45%
29
Typical ALL presentation
``` Bone marrow failure Bone joint pain Raised White cell count Infection Sweats ```
30
Name the most common form of leukaemia
Chronic lymphocytic leukaemia
31
What is the male to female ratio in CLL cases?
M:F 2:1
32
What is required to diagnose CLL?
A lymphocyte count greater than 5
33
Describe the presentation of CLL
``` Typically asymptomatic Bone marrow failure Lymphadenopathy Splenomegaly Fever and sweats ```
34
Less common symptoms of CLL
hepatomegaly infection weight loss
35
Associated findings in CLL
Immunoparesis | Hemolytic anaemia
36
What staging system is used in CLL
Binet
37
Describe the stages in Binet Staging system
Stage A - less than 3 node areas Stage B- more than 3 node areas involved Stage C- Stage B + thromocytopenia or anaemia
38
Indications for CLL treatment
``` Progressive BM failure Progressive splenomegaly Massive lymphadenopathy Lymphocyte doubling time < 6 months or > 50% increase over 2 months Systemic symptoms Autoimmune cytopenias ```
39
Typical management CLL
``` Watch and wait Cytotoxic chemotherapy – fludarabine, Monoclonal antibodies - Rituximab Novel Bruton TKI, PI3K I, BCL-2 inhibitor ```
40
Poor indicators for CLL prognosis
``` Advanced disease (Binet B or C) Atypical lymphocyte morphology Rapid lymphocyte doubling time - in less than 12 months CD38 marker Loss/mutation p53; del 11q23 (ATM gene) Unmutated IgVH gene status ```
41
Presentation of Lymphoma - 4 features
Lymphadenopathy Extranodal disease Systemic B symptoms Bone marrow involvement
42
Staging of lymphoma
Lymph node biopsy CT Bone marrow aspirate Trephine
43
Describe the stages of lymphoma
Stage I - one nodal area Stage II- more than 1 node area Stage III- disease confined to lymph nodes but both sides of diaphragm Stage IV - Extranodal
44
2 elements used to classify Non-Hodgkin Lymphoma
Lineage | Grade
45
What is the most common lineage in Non-Hodgkin lymphoma?
B cell | Rearrangement of Ig
46
Which grade is more aggressive in non-hogkins?
High grade
47
Which grade is incurable, doesn't respond to chemotherapy?
Low
48
What is the most common NH lymphoma?
Diffuse B cell high grade lymphoma
49
Second most common NH lymphoma?
Follicular - low grade
50
Management of follicular lymphoma
Watch and wait | Monitor for spread to Bone Marrow
51
Treatment for Diffuse B cell High grade and follicular lymphoma
AntiCD20 (Rituximab) and chemo combo
52
Describe the trend in age of diagnosis with Hodgkin's lymphoma
Bimodal curve 2 peaks 15-35 yrs Elderly
53
What virus is associated with the second age group that is diagnosed with Hodgkin's lymphoma?
Epstein barr virus
54
How is Hodgkin's lymphoma treated?
Combination chemotherapy ABVD +/- radiotherapy AntiCD30 Monoclonal antibodies Immunotherapy - checkpoint inhibitors
55
How is response to treatment assessed in Hodgkin's lymphoma?
PET scanning
56
What drugs are used in ABVD chemotherapy?
Adriamycin Bleomycin Vinblastine Dacarbazine