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Leukaemia > Acute Leukaemia > Flashcards

Acute Leukaemia Flashcards

0
Q

3 broad classifications of acute leukaemia?

A

Acute Lymphoblastic Leukaemia
Acute Myeloid Leukaemia
Mixed Phenotype (very rare)

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1
Q

What is the definition of acute leukaemia?

A

The presence of >20% blasts in peripheral blood or bone marrow.
Blasts usually comprise <5% of total bone marrow cellularity.

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2
Q

Non-specific presenting features of Acute Leukaemias?

A 
Unwell
Tired
General aches and pains (bone pain in kids)
Fever
Bleeding, sepsis, pallor
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3
Q

Specific presenting features of Acute Leukaemia?

A 
Bone marrow infiltration:
- anaemia
- bleeding tendency (thrombocytopenia)
- infections in mouth and elsewhere (neutropenia)
Tissue Infiltration:
- gum hypertrophy
- lymphodenopathy
- splenomegaly
- CNS disease (more common in ALL)
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4
Q

What happens in ALL and what is it characterised by?

A

Abnormal increase in number of lymphoblasts.
Characterised by rapid onset and progression of signs and symptoms of bone marrow failure (fatigue, fever, infection, bleeding)

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5
Q

What signs and symptoms may be present with ALL other than those which it is characterised as presenting and progressing with?

A 
Bone and joint pain
Enlargement of:
- lymph nodes
- liver
- spleen
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6
Q

What group of people does ALL chiefly affect?

A

Children.

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7
Q

What happens in AML and what is it characterised by?

A

Abnormal increase in number of myeloblasts, especially in bone marrow and blood.
Characterised by rapid onset and progression of symptoms as per ALL.

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8
Q

What group of people does AML effect?

A

Children or adults.

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9
Q

Which is the most common secondary leukaemia?

A

AML

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10
Q

What 3 tests can be used in the initial diagnosis of acute leukaemias?

A

Full blood count.
Differential count (% of WBC’s)
Morphologic review of slide (may show blasts or suspicious cells –> bone marrow biopsy)

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11
Q

Tests used to definitively diagnose acute leukaemias?

A

Bone marrow aspirate.

Bone marrow trephine biopsie.

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12
Q

Morphology of cells in AML?

A

Large blasts.
Primitive nuclei.
Granules.
Auer rods.

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13
Q

Morphology seen in ALL?

A

Scanty cytoplasm.
Primitive nuclei.
No granules.
Granules.

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14
Q

Cytochemistry of AML?

A

Sudan black B +

MPO +

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15
Q

Cytochemistry in ALL?

A

Sudan black B -

MPO -

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16
Q

Flow cytometry for AML?

A

CD13

CD33

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17
Q

Flow cytometry for ALL?

A

B - CD10, CD19

T - CD7, CD3

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18
Q

Cytogenetics in AML?

A

t(8;21)
t(15;17)
inv(16)

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19
Q

Cytogenetics in ALL?

A

t(9,22)

t(4;11)

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20
Q

Additional tests that can be used to diagnose acute leukaemia?

A 
Lumbar puncture to exclude CNS disease.
HIV test.
DIC screen - coagulation tests.
Electrolytes and renal function. 
Acute promyelolytic leukaemia.
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21
Q

What does MPO positive and negative mean?

A

Myeloperoxidase stain is used to test for myeloperoxidase (within granules of myeloid cells).
Used to confirm an acute myeloid leukaemia.
Only 3% possitivity required.

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22
Q

With regards to immunophenotyping, what surface markers are blast cells positive for?

A

CD34.

HLA-DR.

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23
Q

With regards to immunophenotyping, name two myeloid antigens.

A

CD13

CD33

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24
Q

With regards to immunophenotyping, name two B-cell antigens seen in ALL.

A

CD10

CD19

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25
Q

With regards to immunophenotyping, name two T-cell antigens seen in ALL.

A

CD7

CD3

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26
Q

Outline the supportive therapy needed in the treatment of acute leukaemias.

A

Anaemia- filtered irradiated red cells to maintain Hb levels.
Thrombocytopenia - platelet transfusions if indicated.
Prevention of gout and tumour lysis syndrome- hydration and allopurinol.
Prevention of nausea- anti-emetics.
Prevention of infections.

27
Q

Outline the prevention of infections when treating someone with an acute leukaemia.

A

Nursed in single room in special unit.
Regular hand-washing by staff and visitors.
Avoid possible points of contact with infectious agents.
Fresh fruuit and raw vegetables not allowed.
Antibiotic and antifungal prophylaxis.
Attention to mouth care–> prevent oral infections.
Care of intravenous lines.

28
Q

Why is treatment of infections in neutropenic patients an emergency?

A

Infections in such patients can be rapidly fatal.
Immediate empiric antibiotics.
Anti-fungal treatment if no response in 72 hours.
Blood cultures, sputum and urine samples.

29
Q

What is acute leukeamia treated with and what is the aim of such a treatment?

A

Induction therapy, a course of chemotherapy given to induce remission.

30
Q

What is remission? Does it mean one is cured?

A

Where the leukaemia is no longer detectable in the peripheral blood and bone marrow. It does not imply cure?

31
Q

Is one or many drugs used in chemotherapy.

A

Usually a combination of drugs.

32
Q

What is consolidation therapy?
What does it aim to do?
What agents are used?

A

Further courses of chemotherapy given once remission is achieved.
It aims to eradicate the leukaemia.
May contain the same or more intense chemotherapy than induction.

33
Q

What is high dose intensification therapy?

A

Bone marrow transplant.

34
Q

What is maintenance therapy?
How is it given and what is used?
What is the point of such therapy?
Which kind of acute leukaemia is it used for mostly?

A

Less intensive, often oral chemotherapy, as an outpatient.
Usually given monthly.
Given to maintain remission.
Used mostly on ALL.

35
Q

Another name for a bone marrow transplant?

A

Stem cell transplant.

36
Q

When are bone marrow transplants done?

A

To eradicate aggressive cancers, leukaemias, lymphomas, solid tumours.
Also to save a patient treated with high doses of chemotherapy and on occasion radiotherapy.

37
Q

What is an autologous bone marrow transplant?

When can it be done?

A

Where the persons own stem cells are used for the transplant.
Doctors must make sure that cancer has not spread to bone marrow or that it has been successfully treated before the collection of stem cells to reduce chances of relapse after the transplant.

38
Q

What is an allogenic bone marrow transplant?

A

Wherethe stem cells used are rom a matched donor.
Can be a sibling but can be harvested from an unrelated donor.
Carries more risk and is more complicated?

39
Q

What is tumour lysis syndrome?

A

Overwhelming of homeostasis due to:

  • hyperuricaemia
  • hyperkalaemia
  • hyperphosphataemia
  • hypocalcaemia
  • uraemia
40
Q

What is tumour lysis syndrome a result of? What is released?

A 
Excessive destruction of dividing cells (due to chemotherapeutic agents). 
Dead cells release:
- nucleic acids
- intracellular ions
- proteins and their metabolites
41
Q

Complicatioons of chemotherapy?

A

Tumour lysis syndrome.
Normal bone marrow and cells in GIT also killed.
Mucositis - inflammation of mouth, GIT
Nausea
Immune suppression (predispose to infection)

42
Q

What can hyperkalemia lead to?

A

Arhythmias.

Neuromuscular irritability.

43
Q

What can hyperphosphataemia lead to?

A

Precipitation of crystals –> acute renal failure.

44
Q

What can release of large amounts of nucleic acids lead to?

A

Increaded purine metabolism-> hyperuricaemia-> precipitation of crystals–> acute renal failure.

45
Q

What risk factors negatively affect the prognosis of one recieving chemotherapy with respect to ALL?

A 
Age>35
High white cell count at diagnosis
CNS disease at presentation
t(9;22) t(4;11)
If it takes more than 4weeks of weekly chemotherapy to achieve remission.
46
Q

What factors affect the prognosis of one recieving chemotherapy with respect to AML?

A 
Cytogenetics of malignant cells ( good prog t(8;21), bad prog -7)
Performance status of patient.
Age
Primary vs secondary AML
WCC at diagnosis
Response to induction chemotherapy.
47
Q

What is Burkitt Lymphoma?

A

A highly aggressive mature B cell neoplasm with extremely short doubling time and high tumour burden.

48
Q

What signs may Burkitt Lymphoma present with?

A

Lymphodenopathy.
A mass.
Extranodal involvement Eg) meninges
Acute Leukaemia.

49
Q

How to diagnose Burkitt Lymphoma?

A

Morphology
Immunophenotyping
Genetic analysis
(As for acute leuk)

50
Q

What translocation is associated with Burkitts Lymphoma/leukaemia?
Is it specific or only characteristic?

A

t(8;14) - seen in 80% of cases.

51
Q

Which virus has been implicated in the pathogenesis of Burkitts Lymphoma/Leukaemia?

A

EBV.

52
Q

List the three distict clinical forms of Burkitt Lymphoma.

A 
Endemic BL (African)
Sporadic BL (non-endemic)
Immunodeficiency-associated BL
53
Q

Whered doe endemic Burkitt Lymphoma occur?
How does it present?
Who are affected?
What is a characteristic of neoplastic cells in this condition?

A

Equatorial Africa (30-50% childhood cancers)
Often presents as a jaw/facial mass
Peak incidence in 4-7yr age group
EBV genome found in majority of neoplastic cells.
*generally does not manifest as acute leukaemia

54
Q

Where does sporadic BL occur?
Which age group is it most common in?
How does it commonly present?

A

Occurs world-wide.
Mainly in children and young adults?
Commonly presents as a massive abdominal mass with ascites.
*may present as a retroperitoneal mass with spinal cord compression.

55
Q

Who is immunodeficiency-associated BL seen in?
What are some signs and symptoms?
Therapy?

A

Primarily seen in association with HIV infection.
In HIV BL affects those with CD4<200.
S+S of advanced stage:
- lymph node, bone marrow and CNS involvement
*usually chemosensitive, high risk of tumour lysis syndrome.

56
Q

How is Burkitt Lymphoma different to Burkitt Leukaemia?

A

Burkitt Leukaemia –> leukaemic presentation with extensive bone marrow and blood involvement.
But, Burkitt Lymphoma and Burkitt Leukaemia are classified as different manifestations of the same disease.

57
Q

Morphology seen in Burkitt lymphoma/leukaemia?

A

Monomorphic, medium sized cells.
Deeply basophillic cytoplasm with lipid vacuoles.
Round nuclei with finely clumped/dispercsed chromatin and multiple nucleoli.

58
Q

What is associated with a poor prognosis with respect to Burkitt Lymphoma/Leukaemia?

A

Bone marrow and CNS involvement.
Tumour size >10cm.
High serum LDH.

59
Q

Treatment for Burkitt Lymphoma/Leukaemia?

A

Very high dose chemotherapy (higher doses than for ALL and AML)

60
Q

Outline the early management of Acute leukaemia and Burkitt Lymphoma/Leukaemia?

A

Early management is supportive care:

  • use of antibiotics if fever is present
  • transfusion if necessary
  • management of coagulation and metabolic abnormalities
61
Q

How is the Sudan Black Stain useful?

A

In differentiating haematological disorders Sudan black will stain myeloblasts and not lymphoblasts.

62
Q

What is the non-specific esterase stain used for in AML?

A

This stain is used to identify a monocytic component in AML’s and to distinguish poorly differentiated monoblastic leukaemia from ALL.

63
Q

In which disease processes does one see Auer rods?

A

AML

High-grade myelodysplastic syndromes and myeloproliferative neoplasms

64
Q

Chromosomal translocation in acute promyelocytic leukaemia?

A

t(15,17)

Leukaemia (4 decks)

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