HAEM: Lymphoma and CLL Flashcards

1
Q

Where is the BCR-ABL translocation seen?

A

Myeloid neoplasms

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2
Q

What is the MOA of Ibrutinib and Idelasilib?

A

BCR kinase inhibitors

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3
Q

What is the MOA of venetoclax?

A

BCL2 inhibitor = causes apoptosis of CLL cells

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4
Q

What are smear cells characteristic of?

A

CLL

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5
Q

What is the complication that results from extra mutations gained in CLL?

A

Richter transformation –>high grade lymphoma

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6
Q

What CD may be present on immature B cells? What about mature B cells?

A

CD5 - immature

CD19 - mature

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7
Q

Which mutation causes BCL2 overexpression in follicular NHL?

A

t(14;18) –> anti-apoptosis

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8
Q

Does IGHV mutated CLL indicate good prognosis? Why/why not?

A

IgHV mutated indicates good prognosis because it means that the cell has undergone development in the germinal centre

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9
Q

Name a T cell lymphoma of the skin.

A

Mycosis fungoides

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10
Q

Is NHL or HL more common?

A

80% NHL

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11
Q

Which process allows immune system to have diversity in Ig?

A

(1) VDJ recombination,

(2) somatic hypermutation and class switching

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12
Q

What enzyme is involved in somatic hypermutation of Ig?

A

Adenosine induced deaminase (AID)

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13
Q

Give 3 locations for MZL (B cell).

A

Stomach - H.pylori
Salivary glands - Sjogren’s
Thyroid -3 causes

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14
Q

Give a location for T cell MZL.

A

EATL - coeliac

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15
Q

What are 3 causes of thyroid MZL.

A

Riedel’s - IgG4
Hashimoto’s
de Quervain’s - viral

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16
Q

Which type of T cell NHL is caused by a virus?

A

HTLV1 –> adult T-cell leukaemia lymphoma (ATLL)

*usually Caribbean/Japanese

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17
Q

Which type of B cell lymphoma is caused by a virus?

A

EBV –> PTLD (post-transplant lymphoproliferative disorder)

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18
Q

Does HIV cause lymphoma?

A

Increases risk of B cell lymphoma x60

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19
Q

What are the areas of a lymphoid follicle from the centre outwards?

A

GC –> mantle zone –> marginal zone –> paracortical

Between the B cell follicles are T cell areas

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20
Q

What does each layer of the lymphoid follicles contain?

A
GC = B cells and APCs
Mantle = naive B cells
Paracortical = T cell zone
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21
Q

Which lymphoid neoplasm is associated with small round cells? What about large cells?

A

CLL = small round

High grade lymphoma = large

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22
Q

Cyclin D1 expression = ?

A

Mantle cell lymphoma

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23
Q

t(11;14) = ?

A

Mantle cell lymphoma

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24
Q

t(2;5) = ?

A

Anaplastic large cell lymphoma (prognostic factor)

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25
Q

Name 3 low grade/indolent B cell NHLs.

A

Follicular
CLL a.k.a. small lymphocytic lymphoma
Mantle zone lymphoma (MALT)

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26
Q

Name 2 high grade B cell NHLs.

A

DLBCL

Mantle zone lymphoma

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27
Q

Name an aggressive B cell NHL.

A

Burkitt’s

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28
Q

t(14;18) = ?

A

Follicular lymphoma –> BCL2

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29
Q

Where does follicular lymphoma originate? What CD is it associated with?

A

Germinal centre
CD10
BCL-6+ve

30
Q

Where does CLL originate? What CD is it associated with?

A

Naive or post-germinal centre
CD5+ve
CD23+ve
CD19-ve

May be SmIg and CD79 +ve/-ve

31
Q

Where does marginal zone lymphoma originate?

A

Extra-nodal sites by chronic antigen stimulation

32
Q

Where does mantle cell lymphoma originate? What CD is it associated with?

A

Mantle zone origin i.e. pre-GC
CD5 aberrant
Cyclin D1 overexpression

33
Q

Where does Burkitt’s lymphoma originate? What mutations is it associated with?

A

Germinal centre origin

C-myc translocation (4:14, 2:8, 8:22)

34
Q

Which 3 mutations is C-myc in Burkitt’s associated with?

A

4: 14,
2: 8,
8: 22

35
Q

What is the origin of DLBCL?

A

GC or post-GC

NB: GC phenotype = good prognosis

36
Q

What is seen on histopathology in DLBCL?

A

Sheets of large lymphoid cells

37
Q

What mutation confers poor prognosis in DLBLC?

A

p53 +ve

and high proliferation

38
Q

Which cell type is T cell lymphoma commonly associated with?

A

eosinophils

39
Q

Name 4 T cell lymphomas.

A

Anaplastic large cell
ATLL
EATL
Cutaneous

40
Q

What is seen on histology in anaplastic large cell lymphoma?

A

Large “epithelioid” lymphocytes

Might not recognise the T cells

41
Q

What mutation is associated with anaplastic large cell lymphoma?

A

t(2:5)

42
Q

What protein is expressed in anaplastic large cell lymphoma? What does this indicate?

A

Alk-1 = better prognosis

43
Q

What are the types of HL?

A
Classical...
-sclerosing
-mixed cellularity
-lymphocyte rich
-lymphocyte depleted 
Nodular lymphocyte predominant (NLPHL)
44
Q

What is the localisation of HL vs NHL?

A

HL spreads locally, NHL is found at multiple distant sites

45
Q

What CD are found in classical HL? What is the origin of classical HL?

A

CD30+ve
CD15+ve
CD20-ve

GC/post-GC origin

46
Q

What is seen on histopathology in classical HL?

A

Reed-Sternberg

Hodgkin cells with eosinophils

47
Q

What is seen on histopathology in nodular HL?

A

B cell rich nodules

Scattered L&H cells

48
Q

What CD are found in nodular HL?

A

CD20+ve (!) - unlike classical HL
CD30-ve
CD15-ve

49
Q

What weight loss is a significant B symptom in Hodgkin’s lymphoma staging?

A

10% in 6 months

50
Q

Which HL has the best prognosis?

A

Nodular sclerosing - best

Lymphocyte depleted - poor prognosis

51
Q

Which HL subtype is most common in young women?

A

Nodular sclerosing cHL - usually affects neck and mediastinum

52
Q

What is ABVD therapy? What are its advantages over R-CHOP in follicular NHL?

A

A - adriamycin
B - bleomycin
V - vinblastine
D- DTIC

Very effective and maintains fertility. Better than R-CHOP in this way.
But causes cardiomyopathy and pulm fibrosis.

53
Q

Which NHL is antibiotic responsive ?

A

Gastric MALT

54
Q

What is the most common NHL?

A

DLBCL (30%)

Follicular (22%)

55
Q

Can you cure aggressive or indolent lymphomas?

A

Aggressive curable

Indolent not curable

56
Q

How are aggressive lymphomas treated?

A

Like acute leukaemia

57
Q

What prognostic index is used in DLBCL?

A

IPI - international prognostic index

58
Q

What blood investigation is part of the IPI?

A

LDH

IPI = Age, Stage, LDH, Extra-nodal sites. ECOG status

59
Q

What is the treatment regimen for DLBCL?

A

R-CHOP (rituximab-cyclophosphamide-adriamycin-vincristine-prednsolone) with curative aim

60
Q

What prognostic index is used for follicular NHL?

A

FLIPI

61
Q

What is the survival in follicular lymphoma?

A

12-15yrs

62
Q

Is watch and wait acceptable in lymphoma?

A

Yes - if indicated in follicular and CLL only. But not if causing symptoms of compression or pain

63
Q

What is the prognosis with EATL?

A

Generally responds poorly to treatment and is fatal

64
Q

What type of anaemia is seen in CLL?

A

Normocytic normochromic.

65
Q

What are the platelets in CLL?

A

Low

66
Q

What is the immunophenotype of a normal B cell in relation to Cd3/5/19?

A

CD3 -ve
CD5 -ve
CD19 +ve

67
Q

Name 2 prognostic factors in CLL.

A

IgHV mutation status - mutated means better prognosis as cells are mature
Tp53 (17p del or TP53 point mutation) - bad prognosis

68
Q

What staging is used in CLL?

A

Binet(A,B,C) or Rai(0-IV)

69
Q

What prognostic index is used in CLL?

A

IPI for CLL

70
Q

What is the progression with CLL?

A

1/3 don’t progress
1/3 progress but respond
1/3/ progress and don’t respond/die

71
Q

How do you decide when to stop watch and wait and to instead treat CLL?

A

IwCLL criteria

72
Q

Young women with swollen nodes and pain in neck when she drinks. Diagnosis?

A

nodular sclerosing cHL