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Haematology > Lymphoid malignancies > Flashcards

Lymphoid malignancies Flashcards

1
Q

What are primary lymph organs the site of?

A

Primary lymph organs is the site where stem cells can divide and become immunocompetent

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2
Q

What are the secondary lymph organs the site of?

A

• Secondary lymph organs is the site where most of the immune responses occur

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3
Q

What happens to T cells at the thymus?

A

T cells mature in thymus

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4
Q

Where do B cells mature?

A

B cells mature in blood

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5
Q

Example of lymphoid organs

A
  1. Lymph nodes
  2. Spleen
  3. Peyer’s patches
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6
Q

What is the presentation of ALL(Acute lymphoblastic leukemia)?

A

○ Usually non-specific symptoms of bone marrow suppression

○ Symptoms of organ infiltration more often in advanced disease

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7
Q

What is the most commonest leukemia in children?

A

Acute lymphoblastic leukemia

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8
Q

What are the ways to investigate and diagnose acute lymphoblastic leukemia?

A 
○ Bone marrow morphology
○ Immunophenotyping
○ B-cell surface markers
○ Light chain restriction
○ TdT positive
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9
Q

Bone marrow morphology in acute lymphoblastic leukemia?

A

Infiltration by undifferentiated blast cells

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10
Q

Light chain restriction in acute lymphoblastic leukaemia?

A

§ Just one type of light chain produced
instead of different types of

§ light chain, there is just one – due to a mutation

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11
Q

TdT positive in acute lymphoblastic leukaemia?

A

§ At the join some nucleotides randomly removed by exonuclease
§ Some nucleotides randomly added by terminal deoxynucleotidyl transferase (TdT)
§ Cytogenetics

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12
Q

What is the treatment of acute lymphoblastic leukaemia?

A 
○ Chemotherapy
○ Induction
○ Intensification
○ CNS directed chemotherapy
○ Maintenance
○ (Radiotherapy to CNS)
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13
Q

Prognosis in acute lymphoblastic leukaemia of children?

A

> 90% cure

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14
Q

Why do adult have a low survival rate in acute lymphoblastic leukaemia?

A

○ Different cell of origin to children
○ Different oncogene mutations
○ Older patients do not tolerate intensive treatment

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15
Q

What is the presentation of Hodgkin’s lymphoma?

A

Enlarged lymph nodes

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16
Q

What is the possible associated of hodgkin’s leukaemia with?

A

Possible association with epstein barr virus (EBV)

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17
Q

What is EBC known as and due to what?

A

§ EBV known as kissing virus due to saliva transfer

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18
Q

What happens to reed-sternberg cells in Hodgkin’s leukaemia and how’s it spread?

A

○ Reed-sternberg cells typically have bi-lobed nucleus

-Spread in a systemic wa

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19
Q

What is the treatment for Hodgkin’s leukaemia?

A

○ Chemotherapy +/-radiotherapy

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20
Q

What is the prognosis of Hodgkin’s leukaemia?

A

○ 5 year survival ~50-90% depending on age, stage and histology
§ Especially good results in young adults

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21
Q

What are the different grades in non-hodgkins lymphoma?

A

• Different grades
○ Low grade
○ High grade

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22
Q

What cell lymphoma is non-hodgkins lymphoma?

A

T-cell lymphoma

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23
Q

What happens if you get EBV with a non-hodgkins lymphoma?

A

○ If you get EBV, you get glandular fever

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24
Q

What happens to immunosuppressed patients with EBV?

A

• EBV in immunosuppressed patients = bigger problem as immune system cannot fight EBV

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25
Q

What do many lymphomas carry and involving what?

A

• Many lymphomas carry chromosome translocations involving the Ig heavy chain or light chain loci

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26
Q

What are Ig genes highly expressed in?

A

• Ig genes are highly expressed in B-cells

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27
Q

What does each Ig gene have near the constant segment?

A

• Each Ig gene has a powerful tissue specific enhancer near to the constant (C) segment

28
Q

What is the enhancer supposed to produce in order to have an effective immune system?

A

Enhancer is supposed to produce many types of B cells to have an effective immune system

29
Q

What is the normal role of the enhancer?

A

Normal role: activating the promoter of the rearranged V segment

30
Q

Why is it important to have many different types of enhancers?

A

○ The more different types we have, the more likely it is that a foreign antigen will be picked and antibodies against it can be produced

31
Q

What translocations do most cases of follicular lymphoma have?

A

Most cases of follicular lymphoma carry t(14;18)(q32;q31) (READ THIS AS: translocation has happened on Chr14, translocated part has come from Chr18, 32 and 31 have been rearranged)

32
Q

What does the translocation that occurs in follicular lymphoma do and cause?

A

○ This juxtaposes the BCL-2 gene on chromosome 18 with the IgH locus on chromosome 14
○ Causes overexpression of BCL-2 protein

33
Q

What is BCL-2?

A

BCL-2 is an apoptosis inhibitor

34
Q

What does the expression of BCL-2 do and cause?

A

§ Stops any abnormal cells apoptosing

§ These abnormal cells will accumulate and form a lymphoma

35
Q

What is the MYC gene?

A

• Powerful oncogene

36
Q

What is the translocation in some cases of high grade lymphoma?

A

Some cases of high grade lymphoma carry t(18;14)(q24;q32)

37
Q

What does this translocation that is in high grade lymphoma do ? t(18;14)(q24;q32)

A

This juxtaposes the MYC gene on chrom 18 with the IgH locus on chrom 14

38
Q

What other translocations do we get other than t(18;14)(q24;q32)?

A

○ Can also get MYC or BCL-2 translocations to one of the Ig light chain loci

39
Q

What will the MYC gene drive forward?

A

○ MYC will drive forward any cancer/malignant cells that we may have

40
Q

What is the presentation of low grade NHL?

A

○ Enlarged lymph nodes

○ May have night sweats/fever

41
Q

What is the histology of low grade NHL?

A

○ Normal tissue architecture partially preserved
○ Normal cell of origin recognisable
○ Used to name lymphoma- follicular lymphoma, mantle cell lymphoma etc.

42
Q

What can we use for the diagnosis of low grade NHL?

A 
○ Histology
○ Immunocytochemistry
○ Cytogenetics
○ Light chain restriction
○ Light chain restriction
○ PCR
43
Q

What is the treatment for low grade NHL?

A 
○ Chemotherapy
○ Glucocorticoids (e.g. prednisolone)
○ Radiotherapy
○ Monoclonal Ab therapy
○ Rituximab (anti-CD20)
44
Q

What is the prognosis for low grade NHL?

A

○ Relatively indolent (slow-growing)
○ Respond well to therapy
○ But hard to cure

45
Q

What is the presentation of high grade NHL?

A

○ Enlarged lymphnodes

46
Q

What is the histology of high grade NHL?

A

○ Loss of normal tissue architecture

○ Normal cell of origin hard to determine

47
Q

What can we use for the diagnosis for high grade NHL?

A 
○ Histology
○ Immunocytochemistry
○ Cytogenetics
○ Light chain restriction
○ PCR
	§ For clonal Ig gene rearrangement
        § For chromosome translocations
48
Q

What is the treatment used for high grade NHL?

A 
○ Chemotherapy
○ Glucocorticoids
○ Radiotherapy
○ Monoclonal Ab therapy
      § Rituximab- anti CD20
49
Q

What is the prognosis of high grade NHL?

A

○ Variable depending on type, stage and other factors
§ Stages depend on how far it has spread
○ Overall long term survival ~65%

50
Q

What is acute T cell leukaemia/lymphoma associated with?

A

• Associated with retrovirus HTLV-1 (human T-cell leukaemia/lymphoma virus 1) infection

51
Q

What is the presentation of acute T cell leukaemia/lymphoma?

A 
○ Skin infiltration
	§ Sezary syndrome
	§ Mycosis fungoides
	§ Named like this as it looks like fungus growing on the skin
○ Usually CD4 cells
52
Q

What does EBV transform in culture and due to what?

A

• EBV or Human Herpes Virus 4 (HHV4) directly transforms B-lymphocytes in culture
-Due to viral oncogene LMP-1

53
Q

What happens with immunosuppressed individuals with EBV and what develops?

A

○ T cells no longer suppress EBV
○ Endogenous latent EBV may transform B-cells
○ No longer eliminated by cytotoxic T-cells
○ Develop high grade lymphoma

54
Q

What happens to EBV driven lymphoma in transplant patients on the withdrawal of immunosuppression?

A

Lymphoma usually regresses on withdrawal of immunosuppression

55
Q

What happens to lymphoma in on successful HAART in EBV driven immunosuppression?

A

Lymphoma may regress on successful HAART

56
Q

What is the presentation of CLL?

A

○ Most often as incidental finding on fbc
○ Persistent infection(s)
§ Due to immunosuppression
§ Low IgG, suppression of normal B cells
○ Lymph node enlargement
○ Symptoms of bone marrow suppression

57
Q

What is used to diagnose CLL?

A 
○ FBC: Lymphocytosis
○ Immunophenotyping
	§ Cell surface markers
	§ Light chain restriction
○ Cytogenetics
58
Q

What are the 3 aspects of myeloma that give rise to different clinical features?

A
  1. Suppression of normal bone marrow, blood cell and immune cell function
  2. Bone resorption and release of calcium
  3. pathological effects of the paraprotein?
59
Q

What does suppression of normal bone marrow, blood cell and immune cell function do to the patient?

A

○ May become anaemic
○ May have recurrent infections
○ Tendency to bleed

60
Q

What does bone resorption and release of calcium do to the patient?

A

○ Myeloma cells produce cytokines (esp. IL-6)
○ Which stimulate bone marrow stromal cells to release the cytokine RANKL
○ Which activates osteoclasts
§ Lytic lesions of bone
§ Bone pain
§ Fractures
○ Calcium released from bone causes hypercalcaemia
§ Multiple symptoms including mental disturbance

61
Q

What are paraproteins?

A

single monoclonal Ig in the serum

62
Q

What do high levels of paraprotein indicate?

A

High levels indicate malignancy

63
Q

What are the effects of paraprotein?

A

§ Precipitates in kidney tubules cause renal failure

§ Deposited as amyloid in many tissues

64
Q

What do 2% of high paraprotein cases develop into and what does this lead to?

A

○ 2% of cases develop hyperviscosity syndrome
§ Increased viscosity of blood leading to
§ Stroke
§ Heart failure

65
Q

What is used to diagnose myeloma?

A

○ Serum electrophoresis for paraprotein
○ Urine electrophoresis
§ Bence-Jones protein represents free monoclonal light chains
○ Increased plasma cells in bone marrow
○ ESR (very high due to rouleaux formation)
○ Radiological investigation of skeleton for lytic lesions
§ X-ray

66
Q

What is the treatment for myeloma?

A 
○ Chemotherapy (not curative)
	§ Cytotoxic drugs
	§ Glucocorticoids
	§ Thalidomide analogues
	§ Bortezomib
○ Allogeneic bone marrow transplant
	§ Only available for a small number of younger patients (<45yo, not too ill)
	§ Need to find an HLA (MHC) matched donor
         § But potentially curative

Haematology (6 decks)

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