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Y3 immunology and haematology > lymphoproliferative disorders > Flashcards

lymphoproliferative disorders Flashcards

1
Q

what is leukaemia

A

“white blood”

generally used to describe a cancer that you can see in the blood

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2
Q

what is lymphoma and what can they present w/

A

cancers of lymphoid origin

can present w/ lymphadenopathy or extranodal involvement or w/ bone marrow involvement

systemic (B) symptoms: weight loos (>10% in 6mths), fever, night sweats, pruritus, fatigue

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3
Q

diagnosing a leukaemia/lymphoma

A

> 70 different types, defined by the malignant cell characteristics

  • biopsy - tells us what type it is (THIS IS HOW THE DIAGNOSIS IS MADE)
  • clinical examination and imaging - tells us where it is (STAGING)
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4
Q

staging of lymphoma/leukaemia

A

location and extent of the disease

information about prognosis

sometimes influences treatment

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5
Q

lymphocyte differentiation

A
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6
Q

when can malignant conditions occur in the process of lymphocyte differentiation

A

bone marrow: earliest stages of lymphoid cell development e.g. acute lymphoblastic leukaemia

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7
Q

classifications of lymphomas

A

Hodgkin lymphoma is a specific disease

Non-Hodgkin lymphoma is everything else (~70 subtypes)
- divided into high and low grade

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8
Q

key lymphoproliferative disorders (common to least common)

A

Non-Hodgkin lymphoma
- high grade (diffuse large B cell lymphoma), low grade (follicular, marginal zone)

Chronic lymphocytic leukaemia (CLL) - presents in a similar way to low grade NHL

Hodgkin lymphoma

Acute lymphoblastic leukaemia (ALL)

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9
Q

how common are the different lymphoproliferative disorders

A
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10
Q

what is ALL

A

cancerous disorder of lymphoid progenitor cells

no differentiation, instead there is rapid and uncontrolled growth and accumulation

usually in bone marrow but they can go anywhere

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11
Q

normal lymphoid progenitor cells

A

immature

rapidly proliferating cells that differentiate into lymphocytes

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12
Q

what condition is this

A

ALL

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13
Q

how common is ALL

  • incidence
  • age
  • what cell lineage
A

1-2/100 000 pop/yr

75% cases occur in children <6y/o

75-90% cases are of B cell lineage

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14
Q

how does ALL present

A

2-3wk hx of bone marrow failure or bone/joint pain

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15
Q

typical ALL hx

A

17y/o male
1mth impaired vision (both eyes)
1/2 stone weight loss
SOB on minimal exertion

fundoscopy: retinal haemorrhages

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16
Q

blood count for ALL

A

low Hb
high WCC
low plts

bone marrow - 90% B lymphoblasts

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17
Q

what can be seen here

A

normal bone marrow

white circles = fat

normal mix of different types of blood cells and platelets

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18
Q

what can be seen here

A

abnormal bone marrow

hypercellular and uniform cells

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19
Q

ALL cells characteristics

A

large cells

express CD19
CD34, TDT

haven’t had the chance to develop and mature

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20
Q

what is CD19

A

marker expressed by all B cells

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21
Q

what do CD34 and TDT indicate

A

markers of very early immature cells

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22
Q

treatment for ALL

A

induction chemotherapy to obtain remission
consolidation therapy
CNS directed treatment
maintenance treatment for 18mths

stem cell transplantation - if high risk of relapse

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23
Q

newer therapies for ALL

A

bi-specific T cell engagers (BiTe molecules)

CAR T cells

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24
Q

example of bi-specific T cell engagers

A

blinatumumab

25
Q

how do bi-specific T cell engagers work

A

binds to tumour cells (CD19) and activates and recruits pts own T cells to attack the leukaemia cells

26
Q

what are CAR T cells

A

chimeric antigen receptor T cells

27
Q

how are CAR T cells made and used

A

patient/healthy 3rd party T cells harvested

transfected to express a specific T cell receptor expressed on leukaemia cells (CD19)

expanded in vitro

re-infused into patient

can be curative in pts w/ very resistant disease but very expensive

28
Q

key side effects of T cell immunotherapy

A

cytokine release syndrome

neurotoxicity

29
Q

T cell immunotherapy: cytokine release syndrome

A

fever, hypotension, SOB

CAR T cell effect correlated to presence of CRS - sig. number require ITU support

30
Q

T cell immunotherapy: neurotoxicity features

A

confusion w/ normal conscious level

seizure, headache, focal neurology, come

31
Q

ALL poor risk factors

A

increasing age

increased WCC

cytogenetics/molecular genetics: t(9;22), t(4;11)

slow/poor response to treatment

32
Q

ALL outcome

A

adults:
- complete remission rate ~90%
- leukaemia free survival at 5y 30-35%

children

  • 5y overall survival ~90%
  • poor risk pts (slow response to induction or philadelphia +ve) 5y OS 45%
33
Q

what happens to the cells in CLL

A

abnormal cells are mature - usually resemble normal, well behaved lymphocytes

  • grow slowly, or not at all
  • classic example of a low grade condition
  • carry many of the normal markers that B lymphocytes have
34
Q

what does a diagnosis of CLL require

A

lymphocyte count >5 (normal is <4)

35
Q

CLL incidence

A 
>1700 new cases p/a UK
commonest leukaemia worldwide
2M:1F
occasionally familial
rare in far east
36
Q

CLL presentation

A

often asymptomatic

frequently:
- bone marrow failure (anaemia, thrombocytopenia)
- lymphadenopathy
- splenomegaly (30%)
- fever and sweats (<25%)

less commonly:

  • hepatomegaly
  • infections
  • weight loss
37
Q

CLL associated findings

A

immune paresis
haemolytic anaemia
- 20% have +ve direct antiglobulin test
- 8% clinical evidence of haemolytic anaemia

38
Q

what is immune paresis

A

loss of normal immunoglobulin function

39
Q

how is CLL staged

A

Binet staging system

40
Q

CLL - Binet staging

A

A - <3 LN areas, median survival same as age matched controls

B - ≥3 LN areas, ~8yrs med survival

C - stage B + anaemia/thrombocytopenia, ~6yrs

41
Q

CLL indications for treatment

A 
progressive bone marrow failure 
massive lymphadenopathy
progressive splenomegaly 
lymphocyte doubling time <6mths or >50% increase over 2mths
systemic symptoms
AI cytopenias
42
Q

CLL treatment

A

often nothing - watch and wait

cytotoxic chemotherapy
monoclonal antibodies

novel agents

43
Q

cytotoxic chemotherapy for CLL

A

fludarabine

bendamustine

44
Q

monoclonal antibodies for CLL

A

rituximab

obinatuzumab

45
Q

novel agents for CLL

A

less immunosuppressive, oral treatments

Bruton tyrosine kinase inhibitor e.g. ibrutinib
PI3K inhibitor e.g. idelalisib
BCL-2 inhibitor e.g. venetoclax

46
Q

CLL poor prognostic markers

A 
advanced disease (stage B/C)
atypical lymphocyte morphology
rapid lymphocyte doubling time (<12mth)
CD38+ expression
loss/mutation p53, del 11q23 (ATM gene)
unmutated IgVH gene status
47
Q

presentation of lymphoma

A

lymphadenopathy/hepatosplenomegaly
extranodal disease
B symptoms
bone marrow invlolvement

48
Q

assessment/staging of lymphoma

A

LN biopsy
CT scan
bone marrow aspirate and trephine

49
Q

staging of lymphoma

A

I: 1 nodal site of involvement
II: 2 separate nodal sites, either above or below the diaphragm but not both
III: multiple nodal sites, both above and below the diaphragm
IV: extranodal disease involvement

A: absence of B symptoms
B: fever, night sweats, weight loss

50
Q

how is NHL classified

A

lineage: B or T cell (90% B cell origin)
grade: high vs low

51
Q

low grade NHL

A

indolent, often asymptomatic

responds to chemotherapy but incurable

52
Q

high grade NHL

A

aggressive, fast growing
require combination chemo
can be cured

53
Q

specific types of NHL

A

diffuse large B cell lymphoma:

  • commonest subtype of lymphoma
  • high grade

follicular lymphoma:

  • 2nd commonest subtype of lymphoma
  • low grade
  • like CLL, watch and wait if not causing problems
  • both arise from a transformation in the germinal centre of a LN
54
Q

how are diffuse large B cell lymphoma and follicular lymphoma treated

A

combination chemotherapy:

anti-CD20 monoclonal ab + chemo

55
Q

how common is HL

A

30% of all lymphomas

56
Q

age of HL

M vs F

A

bimodal age curve:
15-35y/o
2nd peak later in life

1.9M:1F

57
Q

associations w/ HL

A

EBV

familial and geographical clustering

58
Q

treatment of HL

A 
combination chemo (ABVD - 4 different drugs)
\+/- radiotherapy
monoclonal ab (anti-CD30) - brentuximab
immunotherapy (checkpoint inhibitors)

PET scanning central to assessment of response to treatment

Y3 immunology and haematology (20 decks)

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