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Clin haem > Acute leukaemias > Flashcards

Acute leukaemias Flashcards

AML & ALL

1
Q

what is the dominant clinical feature of acute leukaemia?

A

bone marrow failure caused by accumulation of blast cells

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2
Q

what are the 3 main clinical features associated with bone marrow failure in acute leukaemia?

A
  • anaemia
  • neutropenia which can lead to infections
  • thrombocytopenia which can lead to bleeding
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3
Q

acute leukaemia is normally defined as the presence of at least _____ of blast cells in the bone marrow or blood at clinical presentation.

A

20%
note: it can be diagnosed with less than 20% blasts if certain leukaemia-specific cytogenetic or molecular genetic abnormalities are present

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4
Q

a typical myeloid immunophenotype is ?

A

CD13+, CD33+, CD117, TdT-

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5
Q

Tdt expression defines what kind of lineage?

A

a lymphoid lineage

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6
Q

what are the most common driver mutations promoting clonal expansion in AML?

A

FLT3, NPM1, DNMT3A

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7
Q

what is the immunophenotype found in T-ALL?

A

CD2, cCD3, CD7

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8
Q

what is the immunophenotype found in B-ALL?

A

CD10, CD19, cCD22

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9
Q

Some AML cases are characterized by a gene-fusion event, which usually arises from translocations, with the most common being ?

A

PML-RARA, CBFB-MYH11 and RUNX1-RUNX1T1

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10
Q

AML is the most common form of acute leukaemia in adults and becomes increasingly common with age, with a median onset of 65 years. T or F.

A

True

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11
Q

what are the 6 main groups of AML recognised?

A
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12
Q

what is the characteristic features of the promyelocytic variation of AML?

A

bleeding tendency caused by thrombocytopenia and disseminated intravascular coagulation (DIC)

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13
Q

what are the characteristic features of the myelomonocytic and monocytic subtypes of AML?

A

gum hypertrophy and infiltration, skin involvement (leukaemia cutis) and CNS disease

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14
Q

what are the lab findings/investigations seen in AML?

A
  • normochromic, normocytic anaemia
  • thrombocytopenia
  • neutropenia
  • total white cell count is increased
  • blood films shows blast cells + Auer rods
  • bone marrow is hypercellular with many leukaemic blasts
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15
Q

Acute promyelocytic leukaemia contains which translocation?

A

t(15;17) on the PML and RARA gene which makes the PML-RARA fusion gene

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16
Q

Point mutations in which genes are frequent in AML?

A
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17
Q

What is the general supportive therapy for bone marrow failure (for AML & ALL)?

A
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18
Q

What is the aim of treatment for acute leukaemia?

A
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19
Q

what is the specific therapy for AML?

A
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20
Q

Maintenance therapy is of no proven value except in promyelocytic AML with all-trans retinoic acid (ATRA). T or F.

A

True

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21
Q

Midostaurin inhibits FLT3. T or F.

A

True
note: FLT3 mutations are bad :(

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22
Q

which translocations in AML are the most favourable ones?

A

t(8,21) and inv(16)

23
Q

how is APML treated?

A

it is treated as for DIC with multiple platelet transfusions and replacement of clotting factors with cryoprecipitate or fresh frozen plasma.
also ATRA therapy is given n is combined initially with either arsenic trioxide or anthracycline

24
Q

what is differentiation syndrome and its clinical features?

A
  • aka ATRA syndrome may arise after ATRA treatment
  • clinical problems (cuz of neutrophilia that follows differentiation of promyelocytes) include fever, hypoxia with pulmonary infiltrates and fluid overload
    note: treat with steroids
25
Q

what is complete remission defined as?

A

less than 5% blasts in the bone marrow, normal or near-normal peripheral blood count and no other signs or symptoms of the disease

26
Q

what is the incidence of ALL is highest at which age?

A

3-7 years

27
Q

B-cell lineage rep- resents 85% of cases and these have an equal sex incidence. T or F.

A

True

28
Q

There is a female predominance for the 15% of T-cell ALL. T or F.

A

False
- male predominance

29
Q

Within B-ALL there are several specific genetic subtypes such as what?

A
  • t(9;22) [BCR-ABL1]
  • t(12;21) [ETV6-RUNX1]
  • rearrangements of the KMT2A (MLL) gene
30
Q

About half of the pts with the Ph-like ALL have what kinda mutations?

A

CRLF2 over expression and JAK-STAT pathway mutations
IKZF1 deletions are also common

31
Q

which signalling pathway is activated in most T-ALL cases?

A

NOTCH signalling pathway

32
Q

Early T-precursor (ETP) ALL leukaemia expresses which markers?

A

Blasts in ETP ALL express T-cell marker CD7, but not CD1a and CD8 (these ones are for more mature cells)

33
Q

what are the clinical features of ALL?

A
  • bone marrow failure (ANT)
  • organ infiltration- which can cause tender bones, lymphadenopathy, moderate splenomegaly, hepatomegaly and meningeal syndrome (headaches, diplopia etc.); fundal exam may reveal papilloedema and haemorrhage; fever; testicular swelling or signs of mediastinal compression (T-ALL)
34
Q

what are the lab findings seen/investigations done in ALL?

A
  • normochromic, normocytic anaemia
  • thrombocytopenia in most cases
  • white cell count may be decreased, normal or increased
  • bone marrow is hypercellular with >20% leukaemic blasts
  • biochemical tests may reveal a raised serum uric acid, serum LDH or hypercalcaemia(less common)
  • radiography may reveal lytic bone lesions and a mediastinal mass(characteristic of T-ALL)
35
Q

The risk of tumour lysis syndrome is highest in?

A

children with a high white cell count, T-cell disease or con- current renal impairment at presentation

36
Q

what is minimal residual disease?

A

when small numbers of neoplastic cells/ leukaemic cells are detected by flow cytometry or molecular analysis even when the blood and marrow appear to be clear

37
Q

what are the drugs used in the remission induction in ALL?

A
  • Vincristine
  • L-Asparaginase
  • Corticosteroid (dexamethasone or prednisolone)
    Vaneil likes Camryn
38
Q

what are the drugs used in the consolidation period (to reduce tumour burden to very low levels) for ALL?

A
  • Vincristine
  • Cyclophosphamide
  • Cytosine arabinoside
  • Daunorubicin
  • 6-Mercaptopurine
39
Q

what drugs are used in CNS prophylaxis (to prevent involvement)?

A
  • Intrathecal Methotrexate
  • Cytosine arabinoside
  • Corticosteroid
40
Q

what drugs are used in maintenance therapy in ALL?

A
  • 6-Mercaptopurine
  • Methotrexate
  • Vincristine
  • Corticosteroid
41
Q

how is BCR-ABL1 positive t(9;22) ALL treated?

A

with the use of TKIs like imatinib

42
Q

how is ALL treated in relapsed patients?

A

Chimeric antigen receptor (CAR)-T cell therapy (the patient’s own T cells are programmed to kill B cells expressing CD19) usually followed by allogeneic SCT

43
Q

which features of ALL carry a good prognosis?

A
  • Hyperdiploidy (>50 chromosomes)
  • the t(12;21) ETV6-RUNX1 translocation
44
Q

the presence of MRD in adults after how many months of treatment is an unfavourable prognostic sign?

A

after 3 months

45
Q

which features of ALL carry a poor prognosis?

A
  • hypodiploidy (<44 chromosomes)
  • the Ph translocation t(9;22)
  • most translocations involving 11q23 (MLL)
46
Q

the presence of MRD in children after how many days of treatment is an unfavourable prognostic sign?

A

29 days

47
Q

lumbar puncture for CSF examination is important for what?

A

disease staging

48
Q

differential diagnosis for ALL includes?

A
  • AML
  • aplastic anaemia
  • marrow infiltration by other malignancies
  • infections like infectious mono and pertussis
  • juvenile arthritis
  • immune thrombocytic purpura
49
Q

CAR-T cell therapy is approved for patients with what?

A

refractory B-ALL or large B cell non-hodgkin lymphoma expressing CD19

50
Q

which drug may also help in cases if ALL that express CD22?

A

inotuzumab ozogamicin

51
Q

which ALL drug is a CD19 antibody conjugated to an antibody against CD3?

A

blinatumomab

52
Q

what is necessary after (CAR)‐T cell therapy for relapsed acute lymphoblastic leukaemia?

A

lifelong immunoglobulin replacement therapy

53
Q

what is the most common site of extramedullary relapse in ALL?

A

CNS

54
Q

which marker is most commonly expressed on the surface of blast cells in B-cell ALL?

A

CD19

Clin haem (9 decks)

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