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Haematology > Cancers of Lymphoid Cell Origin > Flashcards

Cancers of Lymphoid Cell Origin Flashcards

1
Q

What is the difference between leukaemia + lymphoma

A

Can be the same thing
Just describes where disease is
Lymphoma = LN
Leukaemia = bone marrow / cells circulating in blood stream

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2
Q

What can cancers of lymphoid origin present with

A 
Lymphadenoapthy
Extranodal
Bone marrow involvement 
B symptoms
Pruritus and fatique
HSM
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3
Q

What are the B symptoms

A

Weight loss >10% in 6 months
Fever
Drenching night sweats

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4
Q

How do you tell what TYPE of cancer is present

A

Biopsy LN
Bone marrow aspirate
Can get leukaemia in LN and lymphoma in bone marrow

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5
Q

How do you tell where the cancer is

A

Clinical examination and imaging (CT)

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6
Q

What types of cancer of lymphoid origin do you get

A 
ALL = lymphoblast (lymphoid progenitor cell) - marrow 
CLL = nodes 
Lymphoma = nodes 
Myeloma = plasma cell - marrow

Will have increased lymphocytes on WCC

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7
Q

What is ALL

A

Neoplastic disorder of lymphoblasts (early lymph stem cells)
Proliferate but do not differentiate
Excessive proliferation cause them to replace other cells leading to pancytopenia
Rapidly fatal
Comes on quickly
CNS involvement + testicular = common
Curable

Can get lymphoblastic lymphoma
- Small lumps of ALL only in LN

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8
Q

Who is ALL common in / other RF

A 
Children 2-6 = most common 
75% cases <6
Down syndrome
RT / X-ray during pregnancy
Infections
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9
Q

Is ALL more common in B or T cells

A

75-90% = B cell

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10
Q

What are the clinical features of ALL

A 
2-3 week history of bone marrow failure 
Bone and joint pain 
- May present just with this = atypical 
B symptoms - weightless, night sweat, fever 
\+- raised WCC 
Infiltration - HSM, lympahdenoaphy, orchidomegaly (common site of recurrence so longer Rx needed in boys) 
CNS involvement - CN palsy / meningism
Abdo pain 
Fatigue
Anorexia
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11
Q

What are the symptoms of bone marrow failure

A

Anaemia
- Fatigue
Thrombocytopenic bleeding / petechiae
Infection due to neutropenia

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12
Q

How do you diagnose ALL

A

IMMEDIATE REFERRAL IF CHILD WITH HEPATOMEGALY / PETECHIAE
If suspect = FBC + film within 24 hours by GP
Bloods - U+E, LFT, clotting, G+S incase of transfusion
Blood film
Bone marrow = diagnostic

Staging
X-Ray to look for infection/ mediastinal and abdominal LN
LP to look for CNS
CT

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13
Q

What does bloods + film show

A 
Leucocytosis
\+- raised WCC or low 
Thrombocytopenia
Low Hb (anaemia) - normocytic 
Increased urate and LDH 
Undifferentiated cells / blasts
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14
Q

What is shown on bone marrow

A

> 20% lymphoblasts

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15
Q

How do you Rx

A

Induction chemo
Can give CNS directed (intrathecal) as high spread
Maintenance chemo 18 months - oral / IV + steroid
Stem cell transplant if relapse
Supportive - blood transfusion, IV fluid, Ax and allopurinol to prevent tumour lysis

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16
Q

What can induction chemo cause

A

Apalsia of bone marrow for three weeks
Reduced T cell immunity
Dose of chemo altered depending on SA, neutrophils and platelets

Also risk of hyperviscosity

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17
Q

What is important to remember with kids

A

Schooling
Growth
Family

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18
Q

What are new therapies for ALL

A

Bispecific T cell engagers (BiTE)

CAR T cells

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19
Q

What do BiTE do

A

Magnet which binds tumour cell to T cell

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20
Q

How do CAR T cells work

A

Harvest patients T cell or donor
Genetically engineer T cell receptor onto cell which matches leukaemia cell marker - CD19
Re-infuse into patient

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21
Q

What are complications of immunotherapy i.e. CAR T

A

Cytokine release syndrome

Neurotoxicity

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22
Q

What is cytosine release syndrome

A

Fever
Hypotension
Dyspnoea
ITU admission

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23
Q

What is neurotoxicity

A 
Confusion
Seizure
Headache
Focal neurology
Coma
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24
Q

What are poor RF for ALL

A 
Increasing age <2 or >10 
Increasing WCC >20 
Philadelphia +Ve (25%)
T or B cell marker
Male 
Non-caucasian 
Orchidomegaly / CNS involvement 
Slow response to Rx
Cytogenetics / immunophenotype
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25
Q

What is prognosis for ALL and how do you follow up

A

Very good
Children = 90% survival
Clinic / PET / MRI

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26
Q

What is CLL

A 
Chronic monoclonal proliferation of well differentiated lymphocytes 
Proliferate without bone marrow failure 
Most common leukaemia in adults 
Most die with it not from it
Low grade NHL
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27
Q

Where do most CLL come from

A

B cells

If in BM
- present anaemia + lymphocytosis
Do get it in LN / spleen

SLL - small lymphocytic lymphoma
- CLL but prefer LN rather than BM
Treated identically

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28
Q

What is seen on bloods + film

A

> 5x10^9 lymphocytes

Spear + smudge cells

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29
Q

What is seen on bone marrow

A

> 30% lymphoblast

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30
Q

What is characteristic immunophenotype of CLL

A

B cell marker

CD5

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31
Q

How does it present

A

Asymptomatic - surprise finding on FBC (increased WCC)
Bone marrow failure if disease is there but rare and late stage
Lymphadenopathy as can live there
HSM
Fever / night sweats / weight loss / anorexia = less common

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32
Q

What type of anaemia

A

Warm autoimmune haemolytic

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33
Q

What is important in relation to the WCC

A

Rate of change rather than value

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34
Q

What if person presents with lymphadenopathy and normal blood count

A

Can still be CLL

Biopsy LN to find out

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35
Q

What are associated findings of CLL

A 
Immune paresis / hypogammaglobinaemia leading to infection 
Haemolytic anaemia 
ITP 
Bone marrow infiltration
Transformation to lymphoma
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36
Q

What is transformation to lymphoma known as

A

Richter’s syndrome

- Suggests by rapid onset of symptoms

37
Q

What happens

A

Enter LN

Change into fast growing high grade NHL

38
Q

How does it present

A 
LN swelling
Fever + no infection
Weight loss
Nasuea 
Night sweats
Abdo pain
39
Q

How do you Dx

A

Bloods + film - smudge cells
Bone marrow
Immunophenotyping

40
Q

How do you stage

A

Binet stage
A = <3LN
B = 3+ LN
C =3+ LN + anaemia + thrombocytopenia

41
Q

What are indications for Rx of CLL

A 
Rat of change of white cells most important not amount 
Progressive bone marrow failure
Massive lymphadenopathy 
Progressive splenomeglay
Rapid lymphocyte doubling time 
Systemic symtpoms
Autoimmune cytopenia
42
Q

What is considered rapid

A

<6 months

>50% increase 2 months

43
Q

How do you Rx

A

Watch and wait
Cytotoxic chemotherapy
Monoclonal Ab
Immunotherapy - Rutiximab

44
Q

What suggests poor prognosis

A 
Stage B or C
Atypical lymphocyte morphology
Rapid doubling time 
Mutation p53 / del11q23
Unmutated IgVH gene status
CD38 expression
45
Q

What is lymphoma

A

Malignant proliferation of lymphocytes which accumulate in LN and other organs
e.g. MALT in B cells in stomach or brain lymphoma

46
Q

How can lymphomas present

A

Nodal disease
Extra-nodal
Systemic Sx
or Both

47
Q

What does nodal tend to be

A

Non-tender
Rubbery
Asymmetrical

48
Q

Likelihood of presenting with nodal disease

A

> 90% of HL

>60% of NHL

49
Q

What are extra-nodal features

A 
Primary central nervous = nerve palsy
Bone marrow involvement
- Normocytic anaemia 
- Eosinophilia
- Increased lDH 
Lung invovlement
HSM 
Gastric = abdo pain / dyspepsia / dysphagia
50
Q

What are systemic Sx

A

B symptoms
Pruritus
Fatigue

51
Q

What are types of lymphoma

A

Hodgkin’s

NHL = everything else (no Reed Sternberg cells)

52
Q

What is Hodgkin’s

A

Specific disease
Characterised by Reed Sternbeg cell
Alcohol induced pain in node

53
Q

What can lymphomas affect

A

B or T cell

54
Q

How do you Dx / stage

A 
FBC + blood film 
Excision LN biopsy 
CT CAP
Bone scan 
Bone marrow biopsy / PET CT to look for involvement
LP 
Bloods - LFT / ESR / LDH / HIV
55
Q

Explain reasoning for bloods

A

LFT - liver mets
ESR - prognosis
LDH = cell turnover
HIV = RF

56
Q

What decides type

A

LN biopsy

57
Q

What is more common NHL or HL

A

NHL

58
Q

How is NHL classified

A

Lineage - B or T cell
Grade - high or low
Histological features

59
Q

What are majority

A

B cell as germinal centre

60
Q

What is most common high grade

A

Diffuse large B cell

61
Q

What is most common low grade

A

Follicular, marginal zone

62
Q

What are RF for NHL

A 
Elderly
Hx viral EBV
FH
Hx chemo / RT
Immunodeficinet - HIV / DM / transplant 
Autoimmune disease - SLE / Sjogren / Coeliac
Obesity
63
Q

What are low grade lymphomas

A

Often asymptomatic

Respond to chemo but incurable

64
Q

What are high grade

A

Aggressive and fast growing
Need combination chemo
Worse prognosis but higher cure rate

65
Q

How is lymphoma graded

A 
Ann-Arbor
1 = one node
2 = 1+ node same side
3 = one node either side 
4 = extra nodal 
A = no B symptoms
B = B symptoms
66
Q

What are complications of NHL

A

Bone marrow involvement
SVC obstruction
Spinal cord compression
Mets

67
Q

What is Burkitt’s lymphoma

A

High grade B cell lymphoma
Fastest growing tumour
Starry sky experience on biopsy

68
Q

What are the two types

A

Endemic - African, involve mandible / Maxilla, strong association EBV

Sporadic - abdo tumour common, HIV RF, mass that can compress

69
Q

What is associated with Burkitt

A

C-myc translocation t(8,14).

70
Q

How do you Rx

A

Chemo

Rapid response which may cause tumour lysis

71
Q

How do you Rx other NHL

A

Chemo / RT
Combination chemo
Targeted therapy - Anti-CD20 Ab (Ritixumab)
Flu / pneumococcal vaccine / Ax prophylaxis
Autologous stem cell transplant if relapse

72
Q

When does Hodkin’s peak

A

Teens

Elderly

73
Q

What is it associated with

A 
EBV
SLE 
Post transplant 
Familial - FH 
Geography
74
Q

Is it curable

A

1st to be cured with chemo

75
Q

What are the features

A 
Lymphadenopathy 
Mediastinal LN may cause bronchial or SVC obstruction
B symptoms
Pruritus
HSM 
Alcohol pain in HL
76
Q

What is seen on bloods (FBC, film, U+E, LFT, LDH, ESR, Ca, urate)

A

Normocytic anaemia
Eosinophilia
Raised LDH
Raised ESR

77
Q

What suggests poor prognosis

A 
B symptoms 
Stage 4
>45
Hb <10.5 or lymphocyte <600
WCC >15000
Raised ESR 
Male
Low albumin <40
Lymphocyte depleted subtype
78
Q

How do you Dx

A

Excision of LN

Bloods

79
Q

How do you treat

A

Combination chemo - ABVD
RT
Monoclonal Ab - anti-CD30
Immunotherapy

80
Q

How do you assess response

A

PET scan

81
Q

What is remission

A

No evidence of leukaemia in blood
Normal or recovering blood
<5% blasts in regenerating marrow

82
Q

Risks of ALL

A 
Neutropenic sepsis 
Hyperurate - allopurinol and fluid 
Tumour lysis 
Hyperviscosity 
Poor growth / development
83
Q

What do you do for infection risk

A

Revaccinate

Ax prophylaxis

84
Q

What should you always have caution with if CHEMO

A

Risk of AVN

85
Q

Background

A

OK

86
Q

Where do you get lymphoid precursor

A

BM
LN / spleen / liver
and everywhere else - gut / lung / brain

87
Q

How does it present

A

BM failure
LN
Splenomegaly
Hepatomegaly

88
Q

Where do plasma cells present

A

Only in bone marrow so tend to present bone disease / anaemia

89
Q

What translocationin Ewings

A

t(11,22)

Haematology (13 decks)

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