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
Q

Poor Risk factors for ALL

A

Increased Age
Increased WBC
Genetics BCR-ABL mutation t(9; 22)
Slow/poor response to treatment

26
Q

Remission rate of adults with ALL

A

90%

27
Q

5 year survival in children with ALL

A

90%

28
Q

5 Year overall survival in at risk patients with ALL

A

45%

29
Q

Typical ALL presentation

A
Bone marrow failure
Bone joint pain
Raised White cell count
Infection
Sweats
30
Q

Name the most common form of leukaemia

A

Chronic lymphocytic leukaemia

31
Q

What is the male to female ratio in CLL cases?

A

M:F 2:1

32
Q

What is required to diagnose CLL?

A

A lymphocyte count greater than 5

33
Q

Describe the presentation of CLL

A
Typically asymptomatic
Bone marrow failure
Lymphadenopathy
Splenomegaly
Fever and sweats
34
Q

Less common symptoms of CLL

A

hepatomegaly
infection
weight loss

35
Q

Associated findings in CLL

A

Immunoparesis

Hemolytic anaemia

36
Q

What staging system is used in CLL

A

Binet

37
Q

Describe the stages in Binet Staging system

A

Stage A - less than 3 node areas
Stage B- more than 3 node areas involved
Stage C- Stage B + thromocytopenia or anaemia

38
Q

Indications for CLL treatment

A
Progressive BM failure
Progressive splenomegaly
Massive lymphadenopathy
Lymphocyte doubling time 
< 6 months or > 50% increase over 2 months
Systemic symptoms
Autoimmune cytopenias
39
Q

Typical management CLL

A
Watch and wait
Cytotoxic chemotherapy – fludarabine, 
Monoclonal antibodies - Rituximab
Novel
Bruton TKI, PI3K I, BCL-2 inhibitor
40
Q

Poor indicators for CLL prognosis

A
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
Q

Presentation of Lymphoma - 4 features

A

Lymphadenopathy
Extranodal disease
Systemic B symptoms
Bone marrow involvement

42
Q

Staging of lymphoma

A

Lymph node biopsy
CT
Bone marrow aspirate
Trephine

43
Q

Describe the stages of lymphoma

A

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
Q

2 elements used to classify Non-Hodgkin Lymphoma

A

Lineage

Grade

45
Q

What is the most common lineage in Non-Hodgkin lymphoma?

A

B cell

Rearrangement of Ig

46
Q

Which grade is more aggressive in non-hogkins?

A

High grade

47
Q

Which grade is incurable, doesn’t respond to chemotherapy?

A

Low

48
Q

What is the most common NH lymphoma?

A

Diffuse B cell high grade lymphoma

49
Q

Second most common NH lymphoma?

A

Follicular - low grade

50
Q

Management of follicular lymphoma

A

Watch and wait

Monitor for spread to Bone Marrow

51
Q

Treatment for Diffuse B cell High grade and follicular lymphoma

A

AntiCD20 (Rituximab) and chemo combo

52
Q

Describe the trend in age of diagnosis with Hodgkin’s lymphoma

A

Bimodal curve
2 peaks
15-35 yrs
Elderly

53
Q

What virus is associated with the second age group that is diagnosed with Hodgkin’s lymphoma?

A

Epstein barr virus

54
Q

How is Hodgkin’s lymphoma treated?

A

Combination chemotherapy ABVD
+/- radiotherapy
AntiCD30 Monoclonal antibodies
Immunotherapy - checkpoint inhibitors

55
Q

How is response to treatment assessed in Hodgkin’s lymphoma?

A

PET scanning

56
Q

What drugs are used in ABVD chemotherapy?

A

Adriamycin
Bleomycin
Vinblastine
Dacarbazine