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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
What is prognosis for ALL and how do you follow up
Very good Children = 90% survival Clinic / PET / MRI
26
What is CLL
``` 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 ```
27
Where do most CLL come from
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
28
What is seen on bloods + film
>5x10^9 lymphocytes | Spear + smudge cells
29
What is seen on bone marrow
>30% lymphoblast
30
What is characteristic immunophenotype of CLL
B cell marker | CD5
31
How does it present
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
32
What type of anaemia
Warm autoimmune haemolytic
33
What is important in relation to the WCC
Rate of change rather than value
34
What if person presents with lymphadenopathy and normal blood count
Can still be CLL | Biopsy LN to find out
35
What are associated findings of CLL
``` Immune paresis / hypogammaglobinaemia leading to infection Haemolytic anaemia ITP Bone marrow infiltration Transformation to lymphoma ```
36
What is transformation to lymphoma known as
Richter's syndrome | - Suggests by rapid onset of symptoms
37
What happens
Enter LN | Change into fast growing high grade NHL
38
How does it present
``` LN swelling Fever + no infection Weight loss Nasuea Night sweats Abdo pain ```
39
How do you Dx
Bloods + film - smudge cells Bone marrow Immunophenotyping
40
How do you stage
Binet stage A = <3LN B = 3+ LN C =3+ LN + anaemia + thrombocytopenia
41
What are indications for Rx of CLL
``` 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
What is considered rapid
<6 months | >50% increase 2 months
43
How do you Rx
Watch and wait Cytotoxic chemotherapy Monoclonal Ab Immunotherapy - Rutiximab
44
What suggests poor prognosis
``` Stage B or C Atypical lymphocyte morphology Rapid doubling time Mutation p53 / del11q23 Unmutated IgVH gene status CD38 expression ```
45
What is lymphoma
Malignant proliferation of lymphocytes which accumulate in LN and other organs e.g. MALT in B cells in stomach or brain lymphoma
46
How can lymphomas present
Nodal disease Extra-nodal Systemic Sx or Both
47
What does nodal tend to be
Non-tender Rubbery Asymmetrical
48
Likelihood of presenting with nodal disease
>90% of HL | >60% of NHL
49
What are extra-nodal features
``` Primary central nervous = nerve palsy Bone marrow involvement - Normocytic anaemia - Eosinophilia - Increased lDH Lung invovlement HSM Gastric = abdo pain / dyspepsia / dysphagia ```
50
What are systemic Sx
B symptoms Pruritus Fatigue
51
What are types of lymphoma
Hodgkin's | NHL = everything else (no Reed Sternberg cells)
52
What is Hodgkin's
Specific disease Characterised by Reed Sternbeg cell Alcohol induced pain in node
53
What can lymphomas affect
B or T cell
54
How do you Dx / stage
``` 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
Explain reasoning for bloods
LFT - liver mets ESR - prognosis LDH = cell turnover HIV = RF
56
What decides type
LN biopsy
57
What is more common NHL or HL
NHL
58
How is NHL classified
Lineage - B or T cell Grade - high or low Histological features
59
What are majority
B cell as germinal centre
60
What is most common high grade
Diffuse large B cell
61
What is most common low grade
Follicular, marginal zone
62
What are RF for NHL
``` Elderly Hx viral EBV FH Hx chemo / RT Immunodeficinet - HIV / DM / transplant Autoimmune disease - SLE / Sjogren / Coeliac Obesity ```
63
What are low grade lymphomas
Often asymptomatic | Respond to chemo but incurable
64
What are high grade
Aggressive and fast growing Need combination chemo Worse prognosis but higher cure rate
65
How is lymphoma graded
``` 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
What are complications of NHL
Bone marrow involvement SVC obstruction Spinal cord compression Mets
67
What is Burkitt's lymphoma
High grade B cell lymphoma Fastest growing tumour Starry sky experience on biopsy
68
What are the two types
Endemic - African, involve mandible / Maxilla, strong association EBV Sporadic - abdo tumour common, HIV RF, mass that can compress
69
What is associated with Burkitt
C-myc translocation t(8,14).
70
How do you Rx
Chemo | Rapid response which may cause tumour lysis
71
How do you Rx other NHL
Chemo / RT Combination chemo Targeted therapy - Anti-CD20 Ab (Ritixumab) Flu / pneumococcal vaccine / Ax prophylaxis Autologous stem cell transplant if relapse
72
When does Hodkin's peak
Teens | Elderly
73
What is it associated with
``` EBV SLE Post transplant Familial - FH Geography ```
74
Is it curable
1st to be cured with chemo
75
What are the features
``` Lymphadenopathy Mediastinal LN may cause bronchial or SVC obstruction B symptoms Pruritus HSM Alcohol pain in HL ```
76
What is seen on bloods (FBC, film, U+E, LFT, LDH, ESR, Ca, urate)
Normocytic anaemia Eosinophilia Raised LDH Raised ESR
77
What suggests poor prognosis
``` B symptoms Stage 4 >45 Hb <10.5 or lymphocyte <600 WCC >15000 Raised ESR Male Low albumin <40 Lymphocyte depleted subtype ```
78
How do you Dx
Excision of LN | Bloods
79
How do you treat
Combination chemo - ABVD RT Monoclonal Ab - anti-CD30 Immunotherapy
80
How do you assess response
PET scan
81
What is remission
No evidence of leukaemia in blood Normal or recovering blood <5% blasts in regenerating marrow
82
Risks of ALL
``` Neutropenic sepsis Hyperurate - allopurinol and fluid Tumour lysis Hyperviscosity Poor growth / development ```
83
What do you do for infection risk
Revaccinate | Ax prophylaxis
84
What should you always have caution with if CHEMO
Risk of AVN
85
Background
OK
86
Where do you get lymphoid precursor
BM LN / spleen / liver and everywhere else - gut / lung / brain
87
How does it present
BM failure LN Splenomegaly Hepatomegaly
88
Where do plasma cells present
Only in bone marrow so tend to present bone disease / anaemia
89
What translocationin Ewings
t(11,22)

Haematology (13 decks)

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