Lymphoid malignancies Flashcards

1
Q

How can lymphomas present?

A

Enlarged lymph nodes (Lymphadenopathy)
Extranodal involvement
Bone marrow involvement
Systemic (B) symptoms - Weight loss >10% in 6 months, fever, night sweats, pruritis, fatigue

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2
Q

How is lymphoma diagnosed?

A

Biopsy to tell us the type

Clinical examination and imaging for staging

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3
Q

Malignancy in which cells causes acute lymphoblastic leukaemia?

A

Lymphoid progenitor/immature lymhpoid cells

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4
Q

Malignancy in which cells cause chronic lymhpoid leukaemia and lymphomas?

A

Mature lymphoid cells - B cells and T cells

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5
Q

What is non-hodgkin lymphoma?

A

Any lymphoma that isn’t Hodgkin lymphoma

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6
Q

What is ALL?

A

Cancerous disorder of lymphoid progenitor cells

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7
Q

What is the normal pathway of lymphoid progenitors?

A

Immature, rapidly proliferating cells differentiating into lymphocytes

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8
Q

What happens in leukaemia?

A

No differentiation, instead, rapid, uncontrolled growth and accumulation

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9
Q

Where does ALL usually present?

A

Usually in bone marrow but can be anywhere

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10
Q

How does ALL present?

A

2-3 week history of bone marrow failure or bone/joint pain

Weight loss

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11
Q

What is the most common form of ALL?

A

B-cell lineage

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12
Q

What blood counts would you expect from ALL?

A

Very low haemoglobin
Extraordinarily high white cell count
Low platelets

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13
Q

What are characteristics of malignant cells in ALL?

A

Large cells
Express CD19 marker -all B-cells have this
Express CD34 and TDT - markers of very early, immature cells

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14
Q

What is standard treatment for ALL?

A

Induction chemotherapy to obtain remission
Consolidation therapy
CNS directed treatment
Maintenance treatment for 18 months
Stem cell transplantation if high risk - adult patient
Bi-specific T-cell engagers (BiTe molecules) - blinatumumab
Chimeric antigen receptor T-cells

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15
Q

What is Chimeric antigen receptor T-cells treatment?

A

Patient/healthy 3rd party T-cells harvested
Transfected to express a specific T-cell receptor expressed on leukaemia cells (CD19)
Expanded in vitro
Re-infused into patient

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16
Q

What are key side effects of CAR T-cell treatment?

A

Cytokine release syndrome - fever, hypotension, dyspnoea

Neurotoxicity

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17
Q

What are ALL poor risk factors?

A

Increasing age
Increased white cells
Cytogenetics/molecular genetics
Slow/poor response to treatment

18
Q

What is Chronic lympoid leukaemia?

A

Mature lymphoid cells, grows slowly, requires a lymphocyte count >5

19
Q

How does CLL typically present?

A
Often assymptomatic at presentation
Bone marrow failure - anaemic/thrombocytopenic
Lymphadenopathy
Splenomegaly
Fever and sweats
Immune paresis
Haemolytic anaemia
20
Q

What is immune paresis?

A

Loss of normal immunoglobulin production

21
Q

What staging system is used for CLL?

A

Binet system

22
Q

What is stage A CLL?

A

<3 lymph node areas

23
Q

What is stage B CLL?

A

3 or more lymph node areas

24
Q

What is stage C CLL?

A

Stage B + anaemia or thrombocytopenia

25
What are 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 ```
26
How is CLL treated?
Often nothing - watch and wait Cytotoxic chemotherapy Monoclonal antibodies Novel agents
27
What are examples of cytotoxic chemotherapy drugs used in CLL?
Fludarabine | Bendamustine
28
What are examples of monoclonal antibodies used in CLL?
Rituximab | Obinatuzumab
29
What are poor prognostic markers for CLL?
``` Advanced disease (Binet B or C) Atypical lymphocyte morphology Rapid lymphocyte doubling time <12 months CD38+ expression Loss/mutation of P53 Unmutated IgVH gene ```
30
What are the 4 stages of lymphoma?
Stage I - One site Stage II - One other site Stage III - Many sites only lymph nodes Stage IV - Extranodal involvement
31
What staging system is used for lymphoma?
Ann Arbor
32
How is non-Hodgkin lymphoma classified?
Lineage - B or T cell | Grade
33
What is low grade lymphoma?
Indolent, often asymptomatic | Responds to chemotherapy but incurable
34
What is high grade lymphoma?
Aggressive, fast-growing Require combination chemotherapy Can be cured
35
What are the 2 commonest lymphomas?
Diffuse large B-cell lymphoma | Follicular lymphoma
36
Is Diffuse large B-cell lymphoma low or high grade?
High grade
37
Is follicular lymphoma low or high grade?
Low grade
38
How are non Hodkin lymphoma treated?
Combination chemotherapy - anti-CD20 monoclonal antibody and chemotherapy
39
Do men or women get Hodkin lymphoma more?
Men
40
How is Hodgkin lymphoma treated?
``` Combination chemotherapy +/- radiotherapy Monoclonal antibodies (anti-CD30) Immunotherapy (checkpoint inhibitors) PET scanning to assess response to treatment ```