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haematology > Lymphoid malignancy > Flashcards

Lymphoid malignancy Flashcards

1
Q

what are the different presentations of lymphoma ?

A

lymphadenopathy
extranodal involvement
bone marrow involvement
systemic (B) symptoms - weight loss, night sweats, pruritus, fatigue

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2
Q

how is lymphoma diagnosed ?

A

biopsy (marrow, node) malignant cell characteristics

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3
Q

how is staging assessed ?

A

clinical exam

imaging - CT

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4
Q

what is the difference between Hodgkins and non-hodgkins ?

A

Hodgkin’s lymphoma is a specific disease

NHL is everything else - 70 subtypes

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5
Q

what are the different types of lymphoproliferative disorders ?

A

NHL -high grade (diffuse large B cell lymphoma), low grade (follicular, marginal zone)
HL
Chronic lymphocytic leukaemia
acute lymphocytic leukaemia

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6
Q

what is the order of how common the lymphoproliferative disorders are ?

A 
high grade NHL
low grade NHL
HL
CLL
ALL
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7
Q

what is acute lymphoblastic leukaemia ?

A

cancerous disorder of lymphoid progenitor cells
no differentiation, rapid, uncontrolled growth and accumulation
usually in bone marrow but can be anywhere

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8
Q

what proportion of cases occur in <6 y/o in ALL

A

75%

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9
Q

what are the presenting symptoms ?

A

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

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10
Q

what is a typical ALL history ?

A

17 male
1 month impaired vision
1/2 stone weight loss
SOB minimal exertion

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11
Q

what investigations for ALL ?

A

FBC - decreased Hb and platelets, elevated WCC

bone marrow aspirate - 90% B-lymphoblasts

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12
Q

what are characteristics of ALL cells ?

A

large cells
express CD19 - all B cells do
CD34, TDT - markers of immature cells

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13
Q

what is the treatment for ALL ?

A

chemotherapy - consolidation, CNS, maintenance for 18 months
stem cell transplant if high risk
newer- Bi specific T cell engagers - blinatumumab
CAR - T cell infusion

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14
Q

what are side effects of T cell immunotherapy ?

A

cytokine release syndrome - fever, hypotension, SOB

neurotoxicity - confusion, seizure, headache, focal neurology, coma

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15
Q

what are risk factors for poor ALL prognosis ?

A

increased age
increased WCC
genetics t(9;22), t(4;11)

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16
Q

what is the prognosis of ALL in adults and children ?

A

adults - complete remission 90%, leukaemia free survival 5y 30-35%
children - 5y survival 90%, poor risk 5y 45%

17
Q

what is different about the CLL cells compared to ALL cells ?

A

grow slowly
low-grade concentration
carry many markers B lymphocytes have

requires a lymphocyte count of >5

18
Q

what is significant about the incidence of CLL ?

A

commonest leukaemia in the world
M2:1F
occasionally familial
rare in Far East

19
Q

what is commonly seen in CLL presentation ?

A

often asymptomatic

bone marrow failure - anaemia, thrombocytopenia
lymphadenopathy
splenomegaly
fever and sweats

less commonly- hepatomegaly, infection, weight loss

20
Q

what are associated findings in CLL ?

A

immune paresis - loss of Ig production

haemolytic anaemia - 20% +ve direct antiglobulin test

21
Q

what is the staging for CLL and what are the stages ?

A

Binet staging
A - <3 lymph nodes, median survival normal
B - =>3 lymph nodes, survival 8 years
C - Stage B + anaemia/thrombocytopenia, survival 6 yrs

22
Q

what are indications for treatment in CLL ?

A 
progressive marrow failure
massive lymphadenopathy
progressive splenomegaly
lymphocyte doubling time <6 months/ 50% over 2 years
systemic symptoms 
autoimmune cytopenias
23
Q

what is the treatment for CLL ?

A

often nothing
chemotherapy - bendamustine
monoclonal antibodies - rituximab
novel - TK inhibitors

24
Q

what are poor prognostic factors for CLL ?

A 
advanced disease B,C
atypical lymphocyte morphology 
rapid lymphocyte doubling time <12 month
CD 38+ expression
loss/mutation p53; del 11q23
unmutated IgVH gene status
25
Q

how does lymphoma present ?

A

lymphadenopathy/ hepatosplenomegaly
extranodal disease
B symptoms
bone marrow involvement

26
Q

how is lymphoma staging assessed ?

A

node biopsy, CT, marrow aspirate and trephine

27
Q

how is lymphoma staged ?

A

1 - single node
2 - multi nodes
3 - nodes, spleen
4 - nodes, spleen, liver, guts

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

28
Q

how are NHL lymphomas classified ?

A

lineage - B or T cells (90% B)

grade - high or low

29
Q

what is low - grade NHL ?

A

indolent, often asymptomatic
responds to chemo
incurable

30
Q

what is high grade NHL ?

A

aggressive, fast growing
require combo chemo
can be cured

31
Q

what are the specific diseases entities for NHL ?

A

diffuse large B-cell lymphoma - commonest, high grade

follicular - 2nd commonest, low grade, leave if no problems

32
Q

how is NHL treated ?

A

combination chemotherapy - anti-CD20 monoclonal antibody + chemo

33
Q

how many lymphomas are HL ?

A

30%

34
Q

what are the two peaks for HL ?

A

15-35

later in life

35
Q

what is HL associated with ?

A

EBV
familial
geographic clustering

haematology (18 decks)

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