Non-Hodgkin's Lymphoma Flashcards Preview

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Flashcards in Non-Hodgkin's Lymphoma Deck (88)
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1
Q

Is NHL common?

A

Relatively

2
Q

How many cases of NHL are there per year in the UK?

A

7500

3
Q

Which gender is NHL more common in?

A

Men

4
Q

What is the median age of presentation for all subtypes of NHL?

A

50 years

5
Q

What are the most common types of NHL seen in children and young adults?

A
  • High-grade lymphoblastic

- Small non-cleaved cell

6
Q

Are low grade NHL more common in the young or old?

A

Old (37% of cases in 35-64, but 16% of cases below 35)

7
Q

What appears to influence the development of NHL in specific areas?

A

Certain geographical factors

8
Q

What kind of NHLs are affected by certain endemic geographical factors?

A
  • Human T-cell lymphotrophic virus-1 (HLTV-1)-associated lymphoma/leukaemia
  • Burkitt’s lymphoma
  • Follicular lymphoma
  • Peripheral T-cell lymphoma
9
Q

Where does HLTV-1-associated T cell lymphoma/leukaemia occur most frequently?

A
  • Japan

- Caribbean

10
Q

Where is Burkitt’s lymphoma more common?

A

Africa, particularly Nigeria and Tanzania

11
Q

Describe the geographical distribution of follicular lymphoma?

A

More common in North America and Europe, but rare in Caribbean, Africa, China, Japan, and Middle East

12
Q

Describe the geographical distribution of peripheral T-cell lymphoma?

A

More common in Europe and China

13
Q

In whom is there a significantly higher incidence of primary CNS lymphoma?

A

Patients with HIV infection and immunosuppression

14
Q

What is important in the pathogenesis of NHL?

A

Non-random chromosomal and molecular rearrangements

15
Q

What does the chromosomal and molecular rearrangements in NGL correspond with?

A

The immunophenotype

16
Q

What is the most common chromosomal finding in NHL?

A

t(14;18)(q32;q21) translocation

17
Q

What does t(14;18)(q32;q21) translocation produce?

A

A juxtaposition of the BCL2 apoptoptic inhibitor oncogene to the heavy-chain region of the immunoglobulin locus

18
Q

What NHL is t(14;18)(q32;q21) translocation most common in?

A
  • Follicular lymphoma (85% of cases have this mutation)

- High grade tumours (28% have it)

19
Q

What chromosomal abnormality is associated with mantle cell lymphoma?

A

t(11;14)(q13;q32)

20
Q

What does the t(11;14)(q13;q32) translocation result in?

A

Over-expression of BCL1

21
Q

What is BCL1?

A

A cell cycle control gene on chromosome 11q13

22
Q

What chromosomal abnormality is frequently seen in high-grade small non-cleaved cell lymphomas?

A

Chromosomal translocations involving 8q24

23
Q

What do chromosomal translocations involving 8q24 lead to?

A

c-myc deregulation

24
Q

What viruses have been implicated in the pathogenesis of NHL?

A
  • EBV
  • HTLV-1
  • HCV
25
Q

What type of NHL is EBV associated with?

A

Burkitts lymphoma

26
Q

What kind of NHL is HTLV-1 associated with?

A

Adult T-cell leukaemia/lymphoma

27
Q

How does HCV increase the risk of NHL?

A

It predisposes B cells to malignant transformation by enhancing signal transduction in binding to CD81

28
Q

What is the classic presentation of NHL?

A
  • Systemic B symptoms
  • Fatigue
  • Weakness
29
Q

What are systemic B symptoms?

A
  • Fever
  • Weight loss
  • Night sweats
30
Q

At what stage can the classical presentation of NHL occur?

A

More common in intermediate- and high grade tumours, but may be present in all stages and tumour subtypes

31
Q

How can low-grade NHL present?

A

Painless, slowly progressive peripheral lymphadenopathy, which sometimes waxes and wanes

32
Q

Can spontaneous regression of enlarged lymph nodes occur in low-grade NHL?

A

Yes

33
Q

What is the problem with spontaneous regression of enlarged lymph nodes in low-grade NHL?

A

Can confuse low-grade NHL with infectious conditions

34
Q

What features are uncommon at presentation in low-grade NHL?

A
  • Primary extranodal involvement

- B symptoms

35
Q

Is the bone marrow involved in low-grade NHL?

A

Frequently is

36
Q

What might the bone marrow involvement be associated with in low-grade NHL?

A

Pancytopenia

37
Q

What % of patients with low-grade NHL have splenomegaly?

A

About 40%

38
Q

How does the presentation of intermediate and high grade NHL differ from low grade?

A

Presents in a more varied manner

39
Q

What do the majority of patients with intermediate or high grade NHL present with?

A

Lymphadenopathy

40
Q

What % of cases of intermediate or high grade NHL have extra-nodal involvement?

A

40%

41
Q

What are the most common sites of extra-nodal involvement in intermediate or high grade NHL?

A
  • GI tract (stomach and small intestine)
  • Skin
  • Bone marrow
  • Sinuses
  • Oral cavity
  • GU tract
  • Thyroid
  • CNS
42
Q

What % of patients with intermediate or high grade NHL have B symptoms?

A

40%

43
Q

How does lymphoblastic lymphoma often present?

A
  • Anterior superior mediastinal mas
  • SVC obstruction
  • Leptomeningeal disease with cranial nerve palsies
44
Q

How do patients with Burkitt’s lymphoma present?

A

Large abdominal mass and symptoms of bowel obstruction

45
Q

What investigations may be done in NHL?

A
  • Careful history and examination
  • Histological evaluation
  • Imaging
  • Bloods
  • Cytogenetic and molecular analyses
  • Examination of CSF
46
Q

What is histologically evaluated in NHL?

A
  • Affected lymph nodes

- Bone marrow

47
Q

How is the affected lymph node tissue for histological evaluation acquired?

A

Excision biopsy of affected lymph node

48
Q

How is bone marrow for histological evaluation acquired?

A

Aspiration and triphine

49
Q

What imaging may be done in NHL?

A
  • CXR
  • CT of chest and abdomen
  • Bone scan
  • PET
50
Q

What is looked for on CXR in NHL?

A
  • Mediastinal or hilar lymphadenopathy
  • Pleural effusions
  • Parenchymal lesions
51
Q

What is looked for on CT of chest and abdomen in NHL?

A
  • Mediastinal, hilar, or parenchymal pulmonary disease
  • Para-aortic or mesenteric lymph nodes
  • Splenomegaly
  • Filling defects in liver and spleen
52
Q

When is bone scan indicated in NHL?

A

If there are MSK symptoms or an elevated alkaline phosphatase

53
Q

What is PET imaging used for in NHL?

A

Determine stage and extent of disease involvement

54
Q

What bloods should be done in NHL?

A

FBC with differential, serum biochemistry, LDH, and ß2 macroglobulin

55
Q

When should HIV serology be considered in NHL?

A

In patients with diffuse large cell, immunoblastic, and small non-cleaved-cell histology

56
Q

When should HTLV-1 serology be considered in NHL?

A

In patients with cutaneous T-cell lymphoma, particularly if they have hypercalcaemia

57
Q

What tissues might cytogenetic and molecular analysis be performed on in NHL?

A
  • Lymph node
  • Bone marrow
  • Peripheral blood
58
Q

When is examination of the CSF required in NHL?

A
  • Diffuse aggressive NHL with bone marrow, epidural, testicular, paranasal sinus, or nasopharyngeal involvement
  • High-grade lymphoblastic lymphoma and small non-cleaved cell lymphomas
  • HIV-related lymphoma
  • Primary CNS lymphoma
59
Q

What are the types of small non-cleaved cell lymphomas?

A

Burkitts and non-Burkitts types

60
Q

What is stage I in NHL?

A

Disease confined to one lymph node or two contiguous lymph node groups

61
Q

What is stage II in NHL?

A

Disease on one side of the diaphragm in two separate lymph node groups

62
Q

What is stage III in NHL?

A

Disease on both sides of the diaphragm

63
Q

What is stage IV in NHL?

A

Extranodal spread with diffuse or disseminated involvement of one or more extralymphatic organs, including any involvement of the liver, bone marrow, or nodular involvement of the lungs

64
Q

What is the problem with treating low grade NHL?

A

They are frequently disseminated at diagosis

65
Q

How is low grade NHL that are stage I with small volume disease managed?

A

Radiotherapy

66
Q

How is low grade NHL that is stage 2-4 managed?

A

Systemic chemotherapy

67
Q

What is the problem with systemic chemotherapy in stage 2-4 low grade NHL?

A

Associated with bone marrow suppression and can result in neutropenic sepsis

68
Q

What is rituximab?

A

A monoclonal antibody

69
Q

What does rituximab do?

A

Targets the CD20 antigen

70
Q

Where is the CD20 antigen found?

A

On the surface of more than 90% of B-cell lymphomas

71
Q

What is rituximab used for?

A
  • Treatment of relapsed low-grade NGL

- Combination with chemotherapy for follicular, mantle cell, and diffuse aggressive NHL

72
Q

How often is rituximab given in relapsed low grade NHL?

A

Weekly for 4-8 weeks

73
Q

What is the response rate to rituximab in relapsed low grade NHL?

A

60%

74
Q

How does the stage at presentation of high-grade and intermediate-grade lymphomas differ to low grade?

A

They are more likely to be confined to one lymph node group (stage 1)

75
Q

What is the result of high and intermediate grade NHL being more likely to be confined to one lymph node group on its management?

A

Radiotherapy can be effectively used

76
Q

What might improve the outcome over radiotherapy in high and intermediate grade NHL?

A

Combination chemotherapy with involved field radiotherapy

77
Q

How are patients with high and intermediate grade large volume stage I NHL managed?

A

Chemotherapy

78
Q

What can patients with poor prognosis lymphoma at presentation or relapse be considered for?

A

High-dose chemotherapy with autologous or allogenic stem cell transplantation

79
Q

What is required for patients having allogenic stem cell transplantation?

A

Long-term immunosuppressio

80
Q

What is the limitation of stem cell transplantation in the treatment of NHL?

A

There is treatment associated mortality of this approach, which may exceed 10%

81
Q

What do lymphoblastic lymphoma have a propensity for?

A

CNS relapse

82
Q

What is the result of the propensity of lymphoblastic lymphoma for CNS relapse?

A

Prophylaxis with intrathecal chemotherapy is incorporated into the chemotherapy regime

83
Q

How long is maintenance therapy given for in lymphoblastic lymphoma?

A

2-3 years

84
Q

Describe the prognosis of disseminated low-grade NHL?

A

They are not cured with treatment, and although 85% of patients achieve a complete remission, this is transient

85
Q

What is the median time of relapse in disseminated low-grade NHL?

A

18 months

86
Q

What is the median time until disseminated low-grade NHL transforms into high grade NGL?

A

7.5 years after diagnosis

87
Q

What is the chance of cure in patients with low-volume stage I high grade tumours?

A

95%

88
Q

What kind of high-grade lymphomas have a worse prognosis?

A
  • Mantle cell

- Burkitt’s