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YEAR 5: Haematology AS > Haem: Lymphoma 2, CLL and Lymphoproliferative disorder > Flashcards

Haem: Lymphoma 2, CLL and Lymphoproliferative disorder Flashcards

1
Q

What are the two main subtypes of Hodgkin lymphoma?

A
  • Classical Hodgkin Lymphoma
  • Nodular lymphocyte predominant Hodgkin lymphoma
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2
Q

What is the fastest growing/aggresive human cancer?

A

Burkitt’s lymphoma

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3
Q

Describe the typical presentation of Non-Hodgkin’s lymphoma.

A

Painless lymphadenopathy

Compression symptoms

B symptoms

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4
Q

Which investigations may be used to stage lymphoma?

A
  • PET scan
  • CT scan
  • Bone marrow biopsy
  • Lumbar puncture (if CNS involvement)
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5
Q

What are some important tests to perform in non-Hodgkin lymphomae and why are they important?

A
  • LDH - marker of cell turnover
  • HIV serology - HIV can predispose to NHL (HTLV1 serology may also be important)
  • Hepatitis B serology - NHL treatment may deplete B cells resulting in fulminant liver failure due to reactivation of hepatitis B in chronic carriers
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6
Q

Broadly speaking, what are the treatment approaches to non-Hodgkin lymphoma?

A
  • Monitor only (in indolent lymphoma)
  • Urgent chemotherapy
  • Non-chemotherapy treatment (e.g. antibiotics to eradicate H. pylori)
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7
Q

What are the two most common types of non-Hodgkin lymphoma?

A

Diffuse large B cell lymphoma (DLBCL)

Follicular lymphoma

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8
Q

List some types of non-Hodgkin lymphoma that are:

  1. Very agressive
  2. Aggresive
  3. Indolent
A
  1. Very agressive
    • Burkitt’s lymphoma
    • T or B cell lymphoblastic lymphoma/leukaemia
  2. Aggressive
    • Diffuse large B cell lymphoma
    • Mantle cell lymphoma
  3. Indolent
    • Follicular lymphoma
    • Small lymphocytic lymphoma (CLL)
    • MALToma
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9
Q

What is the correlation between how aggressive a lymphoma is and how curable it is?

A

The more aggressive it is, the more curable

Indolent lymphoma is more likely to recur

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10
Q

What proportion of NHL are diffuse large B cell lymphoa

A

30-40%

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11
Q

Which factors are taken into account by the international prognostic index (IPI) for lymphoma?

A
  • Age >60
  • High LDH
  • Performance status 2-4
  • Stage III or IV
  • More than one extranodal site
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12
Q

Which chemotherapy treatment is usually used for diffuse large B cell lymphoma?

A
  • R-CHOP
    • Rituximab
    • Cyclophosphamide
    • Doxorubucin
    • Vincristine
    • Prednisolone

NOTE: usually 6-8 cycles

NOTE: achieves a 50% cure rate

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13
Q

What treatment option may be considered for patients with diffuse large B cell lymphoma who relapse?

A

Autologous stem cell transplantation

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14
Q

What proportion of NHL is follicular lymphoma?

A

35%

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15
Q

Which genetic abnormality is associated with follicular lymphoma?

A

t(14;18) - resulting in over-expression of Bcl2 (which is an anti-apoptosis gene)

NOTE: follicular lymphoma is incurable but is indolent

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16
Q

What is the usual first-line treatment approach to follicular lymphoma?

A

Watch and wait

Only treat it clinically indicated (e.g. compression symptoms, massive nodes, recurrent infection)

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17
Q

Which chemotherapy regimen may be used in the treatment of follicular lymphoma?

A
  • R-CVP
    • Rituximab
    • Cyclophosphamide
    • Vincristine
    • Prednisolone
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18
Q

Which lymphoid tissue tends to be affected by marginal zone lymphoma?

A

Extranodal lymphoid tissue (e.g. MALT)

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19
Q

What is the cause of marginal zone lymphoma?

A
  • H. pylori infection - gastric MALToma
  • Sjogren’s syndrome - parotid lymphoma
  • Hashimoto’s thyroiditis - thyroid lymphoma
  • Psittaci infection - lacrimal gland
20
Q

Where is marginal zone lymphoma most commonly seen and how does it tend to present?

A
  • Usually in the stomach
  • Presenting with dyspepsia or epigastric pain
  • Usually Stage 1{E} (E=extranodal)
  • B symptoms are uncommon
21
Q

Outline the process of MALT lymphomagenesis.

A
  • Lymphocytes will respond to H. pylori infection and proliferate
  • At some point, they will over-proliferate and develop cancer-like features but they will still be dependent on antigenic stimulation by H. pylori
  • At this point, treating H. pylori will treat the lymphoma
22
Q

How might gastrict MALToma stage I-II disease be treated?

A
  • Tripy therapy to eradicate H. pylori (2 antibiotics + 1 PPI)
  • Repeat breath test at 2 months
  • Repeat endoscopy every 6 months for 1-2 years then annually

NOTE: failure may require chemotherapy

23
Q

What are the main features of enteropathy-associated T cell lymphoma?

A
  • Mature T cells
  • Involves small intestines
  • Aggressive
  • Caused by chronic antigenic stimulation by gliadin/gluten
24
Q

Describe the typical presenting features of enteropathy-associated T cell lymphoma.

A
  • Abdominal pain/ obstruction/ bleeding/ perforation
  • Malabsorption
  • Systemic symptoms
25
Q

Why is it important to prevent EATL by following a strict gluten-free diet?

A

EATL responds poorly to chemotherapy and is usually fatal

26
Q

What is the most common leukaemia in the Western world?

A

Chronic lymphocytic leukaemia

27
Q

What are the typical laboratory findings in a patient with CLL?

A
  1. Lymphocytosis
  2. Smear cells
  3. Normocytic normochromic anaemia
  4. Thrombocytopaenia
  5. Bone marrow lymphocytic replacement of normal marrow elements

NOTE: it is indolent so is often only picked up on routine blood tests

28
Q

What distinctive antigen phenotype (presence and absence) is suggestive of:

  • Mature B cells
  • Mature T cells
A
  1. Mature B cells:
    • CD19 positive
    • CD5 negative
  2. Mature T cells:
    • CD19 negative
    • CD5 positive
    • CD3 positive
    • CD4 or CD8 positive
29
Q

Which antigen phenotype is suggestive of CLL?

A

CD5+ B cells (i.e. CD19+ and CD5+)

NOTE: this could potentially also be mantle cell lymphoma

30
Q

Which staging system is used for CLL?

A

Rai and Binet

Binet: stages A-C depending on number of lymphoid areas (< or > 3, Hb and platelets)

31
Q

Which laboratory tests are used in CLL to help gauge prognosis?

A

CD38 expression (associated with poor prognosis)

Cytogenetics (FISH)

Immunoglobulin gene mutation status (IgH mutated or unmutated)

32
Q

What are VH genes?

A

The genes that encode the ‘variable heavy’ chain and undergoes somatic hypermutation by VDJ recombination.

33
Q

What is the difference between the VH genes of pre- and post-germinal centre B cells?

A

Pre-germinal centre: VDJ section is unmutated and looks identical to germline

Post-germinal centre: undergone somatic hypermutation so VDJ is mutated and looks different to germline

34
Q

How does VH gene mutations affect prognosis?

A

Unmutated = poor prognosis

Mutated = better prognosis

35
Q

What is an important chromosomal abnormality in CLL that is tested for using FISH?

A
  • Deletion of 17p (Tp53)
  • This is part of the p53 tumours suppressor gene
  • This deletion is associated with a poor prognosis
36
Q

Describe the immunoglobulin levels you would expect to see in CLL.

A

Hypogammaglobulinaemia

Because the malignant B cells are suppressing antibody production by other B cells

37
Q

What is the term used to describe CLL changing into a high grade lymphoma?

A

Richter transformation - 1% risk per year

38
Q

What are some supportive measures used in the treatment of CLL?

A
  • Vaccination (flu, pneumococcus)
  • Infection prophylaxis and treatment (may includ aciclovir, PCP prophylaxis, IVIG)
39
Q

How would autoimmune cytopaenias caused by CLL be treated?

A

Steroids

NOTE: 2nd line is rituximab

40
Q

How would a Richter transformation be treated?

A

R-CHOP

41
Q

What is leukaemia-directed therapy?

A
  • Tailored to the patient
  • Usually involves watching and waiting because it is incurable by chemotherapy
  • Aim to establish remission

NOTE: young people may be cured with allogeneic stem cell transplantation

42
Q

What are some indications for treatment of CLL?

A
  • Progressive lymphocytosis (more than doubling in <6 months)
  • Progressive bone marrow failure
  • Massive or progressive lymphadenopathy/splenomegaly
  • Systemic symptoms (B symptoms)
  • Autoimmune cytopaenias
43
Q

What is the first line treatment for TP53 intact CLL?

A

FCR - Fludarabine, Cyclophosphamide, Rituximab

NOTE: less intensive options may include, rituximab and bendamustine or obinutuzumab (anti-CD20) and chlorambucil (alkylating agent)

44
Q

Under what conditions might CLL be considered high risk?

A

Patients with TP53/17p deletion

Refractory disease or early relapse (<24 months)

45
Q

What are some newer treatment options for high risk CLL?

A

Bruton Tyrosine Kinase Inhibitors - ibrutinib, idelalisib

Bcl2 Inhibitors - venetoclax

CAR-T therapy

46
Q

Describe how CAR-T therapy for CLL works.

A
  • CAR-T are autologous T cells that are modified to contain chimeric antigen receptors
  • The internal part of the receptor is responsible for cell signalling
  • The external part is designed to target CD19 (on B cells) thereby enabling B cell depletion

YEAR 5: Haematology AS (20 decks)

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