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Year 5 path > haematology - leukaemia > Flashcards

haematology - leukaemia Flashcards

1
Q

acute leukaemia (ALL and AML)

A

acute, rapidly progressing, and fatal

neoplastic process affecting blood precursor cells

immature blasts > 20% of bone marrow cells

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2
Q

clinical features of ALL and AML

A

bone marrow function failure

  • anaemia
  • thrombocytopenia (bleeding)
  • neutropenia (infection)

organ infiltration

  • hepatomegaly
  • splenomegaly
  • lymphadenopathy
  • bone pain
  • CNS
  • skin
  • gum hypertrophy
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3
Q

aetiology of ALL and AML

A

most times, no clear trigger

ionising radiation
cytotoxic drugs
benzene

pre-leukaemic disorders
Down’s

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4
Q

Down’s syndrome and leukaemia

A

significantly increased risk of ALL/AML

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5
Q

dx of ALL and AML

A

morphology +/- cytochemistry
immunophenotyping with flow cytometry
cytogenetics
molecular genetics

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6
Q

ALL vs AML epidemiology

A

ALL: childhood
AML: increased risk with age and <2yrs

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7
Q

clinical features of ALL

A 
lymphadenopathy
CNS involvement
testicular enlargement
thyme enlargement
extra medullary disease
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8
Q

what is extra medullary disease

A

solid leukaemia deposits outside the bone marrow

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9
Q

clinical features of AML

A

lymphadenopathy is less common

M3: propane to DIC and bleeding
M4/5: skin/gum infiltration and hypokalaemia

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10
Q

ALL investigations and findings

A 
high WCC (blasts)
blasts have tails/blebs of cytoplasm

flow cytometry:
CD34 = stem cells
CD3, 4, 8 = T cells
CD19, 23 = B cells

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11
Q

AML investigations and findings

A 
high WCC (blasts)
Auer rods and granules

flow cytometry:
CD34 = stem cells
CD33, 13, MPO = myeloid cells

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12
Q

Auer rods

A

seen in AML

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13
Q

philadelphia chromosome leukaemia

A

9:22 translocation

seen in ALL/CML

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14
Q

treatment of ALL/AML

A

chemotherapy

targeted treatments

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15
Q

chemotherapy for ALL

A

remission induction:
chemo agents + steroids

consolidation:
high dose multi drug chemo

maintenance:
F: 2yrs, M: 3 yrs

consider alto-stem cell transplant if high risk of relapse

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16
Q

targeted treatment of ALL

A

nelarabine (T-ALL)
CAR-T cells
blinatummab (B-ALL)
imatinib if 9,22 translocation

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17
Q

imatinib

A

9:22 Philadelphia chromosome ALL

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18
Q

supportive treatment for ALL

A 
blood products
Abx
allopurinol
fluids
electrolytes
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19
Q

chemotherapy for AML

A

remission induction:
daunorubicin
cytarabine

consolidation:
cytarabine

No CNS propylaxis/maintenance

consider allo-SCT if high risk of relapse

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20
Q

ATRA

A

acute promyelocytic leukaemia (AML)

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21
Q

midostaurin

A

FLT3 mutations AML

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22
Q

gemtuzumab

A

CD33 immunotherapy AML

23
Q

enasidenib

A

IDH mutations AML

24
Q

supportive treatment for AML

A 
blood products
Abx
allopurinol
fluids
electrolytes
25
Q

CML

A

myeloproliferative disease

typically 40-60yrs

26
Q

presentation of CML

A

FLAWS
infections
bruising
massive splenomegaly

27
Q

remission rate of CML

A

95% with imatinib

28
Q

investigations for CML

A

PCR for BCR-ABL fusion gene
FISH for Philadelphia Chr
bloods
bone marrow aspirate

29
Q

bloods for CML show

A

high WCC
high neutrophils
high basophils

30
Q

bone marrow aspirate for CML

A

hypercellular

spectrum of immature and mature granulocytic cells

31
Q

phases of CML

A

chronic
accelerated
blast

32
Q

chronic phase of CML

A

<5% blasts in bone marrow/blood

WCC increased over years

33
Q

accelerated phase CML

A

> 10% blasts in bone marrow/blood

increasing manifestations

34
Q

blast phase CML

A

> 20% blasts in bone marrow/blood
resembles acute leukaemia
months +/- FLAWS

35
Q

treatment of CML

A

imatinib
disatinib/nilotinib for resistance

immediately after dx confirmed regardless of symptoms

accelerated phase less responsive

allogeneic SCT for young pts in blast phase

36
Q

action of imatinib

A

BCR-ABL tyrosine kinase inhibitor

37
Q

CLL

A

lymphoproliferative disease
M>F, 65-70yrs
can progress to a form of lymphoma

38
Q

Richter’s transformation

A

progression of CLL to lymphoma

39
Q

clinical features of CLL

A 
may be asymptomatic
symmetrical painless lymphadenopathy
bone marrow failure (anaemia, thrombocytopenia, neutropenia)
FLAWS
hepatosplenomegaly
40
Q

CLL is associated with

A

autoimmunity - Evan’s Syndrome
AIHA
ITP

41
Q

CLL vs small lymphocytic lymphoma

A

same disease process, different presentations

CLL: bone marrow
SLL: lymph nodes

42
Q

investigations and findings for CLL

A

bloods: high WCC with lymphocytosis

low serum immunoglobulin

flow cytometry: monoclonal population - CD5+ CD23+

blood film: smear cells

bone marrow aspirate: abnormal, lymphocytic replacement

43
Q

good prognostic factors for CLL

A

hyper mutated Ig gene
low ZAP-70 expression
12q14 deletion

44
Q

bad prognostic factors for CLL

A

raised LDH
CD38+
11q23 deletion

45
Q

12q14 deletion in CLL

A

good prognostic factor

46
Q

11q23 deletion in CLL

A

bad prognostic factor

47
Q

Staging of CLL

A

Binet staging A, B, C

48
Q

Stage A CML

A

high WCC
<3 groups of enlarged lymph nodes

no treatment required

49
Q

Stage B CML

A

> 3 enlarged lymph nodes

50
Q

Stage C CML

A

anaemia or thrombocytopenia

51
Q

treatment of CML if asymptomatic with slow progressive disease

A

watchful waiting

52
Q

supportive treatment of CML

A

transfusions

infection prophylaxis

53
Q

1st line treatment of CML if p53 deletion

A

alemtuzmab/ibrutinib/idalalisib

transplant

54
Q

1st line treatment of CML if no p53 deletion

A

clinical trial

chlorambucil/fludarabine/rituximab

Year 5 path (16 decks)

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