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review haem > Haem: Lymphoma 2, CLL and Lymphoproliferative disorder > Flashcards

Haem: Lymphoma 2, CLL and Lymphoproliferative disorder Flashcards

1
Q

Describe the typical presentation of lymphoma.

A

Painless progressive lymphadenopathy
* Can cause compression symptoms e.g. dysphagia if compressing oesophagus

Inflitration symptoms - skin rash, visual changes, neurological deficits

Recurrent infection (BM supression)

Constitutional symptoms (B symptoms)

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2
Q

How is lymphoma diagnosed?

A

Biopsy with histological WHO classification of lymphoma subtype

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3
Q

Which investigations may be used to stage lymphoma?

A
  • Imaging - PET, CT, MRI
  • Bone marrow biopsy
  • Lumbar puncture (if CNS involvement)
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4
Q

What are some important tests to perform in lymphoma
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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5
Q

What are the 2 main types of Hodgkin’s lymphoma

A

Classical (95%)

Nodular lymphocyte predominant (5%) - causes recurrent disease in elderly

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6
Q

What is the main type of classical HL and which type has a poor prognosis?

A

Nodular sclerosing is the main type of classical HL (predominantly affects young women aged 20-30)

Lymphocyte depleted HL is the type that has a poor prognosis

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7
Q

What is the chemotherapy regime used in cHL

A

ABVD
* Adriamycin
* Bleomycin
* Vinblastine
* Dacarbazine (DTIC)

Given at 4 weekly intervals for 2-6 cycles (dependant on stage and response)

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8
Q

How is HL relapse treated

A

High dose chemotherapy with PB autologous stem cell transplant

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9
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 in gastric marginal zone lymphoma)
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10
Q

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

A

Diffuse large B cell lymphoma (DLBCL) - 30%

Follicular lymphoma - 22%

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11
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 (marginal zone lymphoma)
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12
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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13
Q

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

A
  • Age
  • Ann Arbor stage
  • LDH
  • Presence of extra-nodal disease
  • ECOG performance status
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14
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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15
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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16
Q

Which genetic abnormality is associated with follicular lymphoma?

A

t(14;18) - over-expression of Bcl-2 (anti-apoptosis gene)

NOTE: follicular lymphoma is incurable but is indolent - median survival 12-15 years

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17
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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18
Q

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

A

R-CVP (rituximab, cyclophosphamide, vincristine, prednisolone)

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19
Q

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

A

Extranodal lymphoid tissue (e.g. MALT)

20
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
21
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
22
Q

Outline the process of MALToma pathogenesis.

A
  • Lymphocytes will respond** chronic antigen stimulation** from 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
23
Q

What are the symptoms of early stage gastric MALToma

A

Epigastric pain, ulceration, bleed

B-symptoms uncommon

24
Q

How might gastric 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

25
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
26
Q

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

A
  • Abdominal pain/ obstruction/ bleeding/ perforation
  • Systemic symptoms
27
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

28
Q

What is the most common leukaemia in the Western world and what is its median age of presentation

A

Chronic lymphocytic leukaemia - proliferation of mature B-cells

Median age of presentation is 72

29
Q

What are the typical laboratory and blood smear 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

30
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
31
Q

Which antigen phenotype is suggestive of CLL?

A

CD19+ and CD5+

NOTE: this could potentially also be mantle cell lymphoma

32
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)

33
Q

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

A

FISH cytogenetic panel

  • TP53 mutation status

IgH V gene mutation status

CD38 expression (associated with poor prognosis)

34
Q

How to TP53 and IgH V gene mutations affect prognosis

A

TP53 - mutation/deletion associated with worse prognosis

IgH V gene - mutation associated with better prognosis

35
Q

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

A

Pre-germinal centre: variable region is unmutated and looks identical to germline

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

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

Leads to increased risk of infection

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 (covid, flu, pneumococcus)
  • Infection prophylaxis and treatment (e.g. PCP prophylaxis)
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 to stop watch and wait and initate treatment of CLL?

A
  • Progressive lymphocytosis (doubling in <6 months or >50% increase in 2 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

What are some newer treatment options for high risk CLL?

A

B cell receptor kinase inhibitors
* Bruton tyrosine kinase inhibitors - ibrutinib
* PI3K inhibitor - idelalisib

Bcl2 Inhibitors - venetoclax (effective but risk of tumour lysis syndrome)

CAR-T therapy

45
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

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