Aani Haem: Lymphomas Flashcards

1
Q

3 commonest sites for lymphomas?

A

Lymph nodes
BM
Blood

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2
Q

From which cell do lymphomas arise?

A

Lymphocytes

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3
Q

other Lymphoid tissue associated with lymphomas?

A

MALT
Spleen
Can occur anywhere e.g. breast lymphoma, CNA lymphoma etc

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4
Q

Why are lymphomas so common?

A

Because lymphocytes have genetic instability

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5
Q

3 Risk Factors for lymphomas?

A
  1. Antigenic stimulation (e.g. infections - H.Pylori etc)
  2. Direct Viral Integration e.g. HTLV1 (Caribbean/Japan) causes Adult T Cell Lymphoma/leukaemia
  3. Immunosuppression
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6
Q

Why are HIV patients more at risk of B cell lymphomas after EBV?

A

Immunosuppressed pts have lost T cell regulatory function

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7
Q

What usually prevents B cell lymphomas when you get EBV?

A

T cells normally attack the infected B cells so they cannot proliferate too much

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8
Q

What is the role of T cells?

A

They regulate B cells via cytokines

Direct cytotoxic function

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9
Q

What percentage of all lymphomas are Hodgkins?

A

20%

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10
Q

Which virus is associated with Hodgkins Lymphoma?

A

EBV

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11
Q

What is the peak incidence of Hodgkins Lymphoma?

A

Bimodal: 20-29 years (most common) and 60+ years

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12
Q

What are the symptoms of Hodgkins?

A
  • Asymmetrical painless lymphadenopathy
  • Pain after alcohol in affected nodes
  • Obstructive symptoms e.g. compression of foodpipe
  • Can get B symptoms (low grade fever/eight loss/sweats)
  • Pel Ebstein Fever in 20% (cyclical)
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13
Q

Which cells are commonly seen in Hodgkin’s?

A

Reed-steinberg Cells (binucleate owl-eyed cells) - seen on a background of lymphocytes & reactive cells

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14
Q

Most common subtype of Hodgkins?

A

Nodular Sclerosing

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15
Q

Subtypes of Hodgkins?

A

Nodular Sclerosing
Mixed cellularity
Lymphocyte depleted
Lymphocyte Rich

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16
Q

How is the staging done for Hodgkins?

A

1 - ONE LN region affected
2 - 2+ LNs on same side of diaphragm
3 - 2+ LNs on opposite side of diagram
4 - Extranodal sites e.g. Live/BM

Add B if they have B constitutional symptoms

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17
Q

What is the management for Hodgkin’s Lymphoma?

A
ABVD chemo
Adriamycin
Bleomycin
Vinblastine
Decarbazine
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18
Q

How many cycles of chemo needed for stage 1-2 Hodgkin’s?

A

2-4 cycles

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19
Q

How many cycles of chemo needed for stage 3-4 Hodgkin’s?

A

6-8 cycles

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20
Q

What is the name for SC transplant that comes from patient’s own body?

A

Autologous

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21
Q

What is the name for SC transplant that comes from HLA-matched donor?

A

Allogenic

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22
Q

How do you treat someone with relapsing Hodgkins?

A

Aggressive Chemo + Autologous SCT

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23
Q

Which lymphoma gives you pain after alcohol?

A

Hodgkin’s Lymphoma (NOT NHL!)

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24
Q

Symptoms of Non-Hodgkin’s Lymphoma?

A
Varies between subtypes.
Generic: Painless lymphadenopathy
Asymmetrical
No pain after alcohol
Constitutional symptoms
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25
Q

What are the types of B-Cell NH Lymphomas?

A
Follicular 
MALT
Diffuse
Mantle
Burkitt's
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26
Q

What is a mnemonic to remember the B-Cell NH Lymphomas in order of indolent to aggressive?

A
Fairies
Make 
Dust
on the Mantle place
Burkitt's (I know that's the most aggressive)
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27
Q

Which is the least responsive to therapy of the B cell NH Lymphomas?

A

Follicular (fairies are innocent/indolent but MIGHT resistant)

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28
Q

Which oncogene is expressed in Burkitt’s lymphoma?

A

C-myc

(MIKE (Myc) BURKITT) - the burkitt monster

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29
Q

Physical symptoms of Burkitt’s?

A

Jaw involvement and abdo mass

30
Q

Describe the histology appearance of Burkitt’s?

A

Starry Sky (Burkitt’s monster comes out at night when the stars are out)

31
Q

What is the translocation in Burkitt’s lymphoma?

A

t(8;14) - B looks like 8.

32
Q

What is the translocation in Mantle Cell lymphoma?

A

t(11;14) (Mantlle ll looks like 11. 11 and 14 cups on the mantle place)

33
Q

What is the translocation in Diffuse Large B Cell lymphoma?

A

t(14;18) (you look DIFF between aged 14 and 18)

34
Q

What is the translocation in MALT/marginal zone lymphoma?

A

t(11;18) - drink MALT at secondary school (aged 11-18)

35
Q

What is the translocation in Follicular lymphoma?

A

t(14;18) (Follicular starts with F). Same as Diff. Cos you get follicles on your face

36
Q

What histology do you see in Mantle Cell lymphoma?

A

Angular Nuclei

37
Q

Why do people in Africa get Burkitt’s lymphoma?

A

Because in places where Malaria is high, P falcuparum wears down EBV resistance

38
Q

What histology do you see in Diffuse Large B cell lymphoma?

A

Sheets of large lymphoid cells

39
Q

What histology do you see in Follicular lymphoma?

A

Nodular/follicular appearance

40
Q

What are 2 causes of MALT/Marginal Zone Lymphoma?

A

Causes by chronic antigen stimulation commonly by:

  • H.Pylori –> gastic MALT lymphoma
  • Sjogren’s Syndrome –> Parotid lymphoma

(TIP: Drink MALT at school t(11;18). It was tasty so you drooled- parotid. But you got a tummy bug sadly - H.Pylori).

41
Q

Which gene is affected in Follicular B Cell NH Lymphoma?

A

BCL 6+ gene (t;14-18)

42
Q

Treatment of Follicular B Cell NH Lymphoma?

A
  1. Wait + Watch

2. Rituximab

43
Q

Treatment of Marginal Zone/MALT NH Lymphoma?

A
  1. Remove antigenic stimulation
  2. Triple therapy for H.Pylori: amoxicillin, clarithromycin, and a proton pump inhibitor such as omeprazole, lansoprazole
44
Q

Treatment of Diffuse Large B-Cell NH Lymphoma?

A
  1. Rituximab - CHOP
    (So diffuse you need to chop it)
  2. Auto-SCT for relapse
45
Q

Treatment of Mantle Cell NH Lymphoma?

A
  1. Rituximab - CHOP
    (So diffuse you need to chop it)
  2. Auto-SCT for relapse
46
Q

Treatment of Burkitt’s NH Lymphoma?

A
  1. Rituximab

2. Auto-SCT

47
Q

Which lymphomas are associated with EBV

A

Hodgkin’s

AND Burkitt’s B-Cell Non-Hodgkins

48
Q

Which cellular protein is increased in Mantle Cell NH Lymphoma?

A

Cyclin D1

49
Q

Increased Cyclin D1 is associated with which lymphoma?

A

Mantle Cell NH Lymphoma

50
Q

Which demographic are most affected by Mantle Cell NH lymphomas?

A

Middle Aged Men (man = mantle cell)

51
Q

What is Rituximab?

A

Anti CD20 Monoclonal Ab. CD 20 is found in B-Cells so Rituximab is used in B Cell NH Lymphomas

52
Q

What percentage of Lymphomas are Non-Hodgkins?

A

80%

53
Q

What are the categories of NH Lymphomas?

A

B-Cell derived or T-Cell derived

54
Q

What will B cells begin to express in NH lymphomas?

A

CD5 and CD23 (which they shouldn’t normally express)

55
Q

What is Alemtuzumab? When is it used?

A

Anti CD52 - used in CLL and T-Cell NH Lymphoma

56
Q

What investigations do you do for Hodgkin’s Lymphoma?

A

CT/PET scan and LN/BM biopsy - especially useful for staging

57
Q

Mnemonic to remember T-Cell NH Lymphomas?

A

Ana’s CAPE:

Anaplastic Large Cell Lymphoma
Cutaneous T Cell Lymphoma
Adult T Cell Lymphoma
Peripheral T Cell Lymphoma
Enteropathy Assoicated T Cell Lymphoma (EATL)
58
Q

Which infection is ass. with Adult T Cell NH Lymphoma?

A

HTLV-1 viral infection

59
Q

Which age group are affected by Anaplastic Large Cell lymphomas?

A

Children & young adults (like Ana)

60
Q

Which protein is seen in 30% of Adult-T Cell NH Lymphomas?

A

Philadelphia Chromosome

61
Q

Which cells are seen in Anaplastic Large T Cell lymphomas?

A

Large Epitheloid cells

62
Q

Which ethnic groups are prone to getting Adult T-Cell Lymphomas?

A

Japanese and Caribbean because HTLV-1 is found there

63
Q

What skin infection is ass. with cutaneous T Cell lymphoma?

A

MYCOSIS FUNGOIDES

64
Q

What condition is ass. with Enteropathy Associated T Cell Lymphoma (EATL)

A

long standing Coeliac Disease

65
Q

Which Hodgkins Lymphoma has the best prognosis?

A

Lymphocyte predominant

66
Q

Which Hodgkins Lymphoma has the worst prognosis?

A

Lymphocyte depleted

67
Q

What are the 2 types of Burkitt’s lymphoma?

A
  1. endemic (African) form: typically involves maxilla or mandible
  2. sporadic form: abdominal (e.g. ileo-caecal) tumours are the most common form. More common in patients with HIV
68
Q

Explain the Starry Sky appearance in Burkitt’s lymphoma?

A

lymphocyte sheets interspersed with macrophages containing dead apoptotic tumour cells

69
Q

What is a side effect of treating Burkitt’s lymphoma with chemo?

A

Tumour lysis syndrome

70
Q

What are complications of tumour lysis syndrome?

A
hyperkalaemia
hyperphosphataemia
hypocalcaemia
hyperuricaemia
acute renal failure