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Final Notes > Paeds: Leukaemia > Flashcards

Paeds: Leukaemia Flashcards

1
Q

Definition

A

Proliferation of immature WBCs (blasts).

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2
Q

Type

A

Vast majority are acute, chronic myeloid leukaemia is very rare. Classified by cell as lymphocytic (lymphoid cells) or myeloid (granulocytic or monocytic).

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3
Q

Clinical features

A

Present due to bone marrow failure with anaemia or bruising, hepato-splenomegaly, lymphadenopathy, infection due to neutropenia or bone pain.
Bone marrow aspiration shows replacement of normal elements by blast cells.

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4
Q

ALL Aetiology

A
  • 80% of leukaemia in children remainder is acute myeloid/acute non-lymphocytic leukaemia (AML/ANNL).
    Chronic myeloid leukaemia and other myeloproliferative disorders are rare.
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5
Q

ALL Clinical presentation

A
  • Peak presentation at 2-5 years
  • Short history (days or weeks)
    Clinical symptoms due to disseminated disease and systemic ill-health from infiltration of the bone marrow or other organs with leukaemic blast cells
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6
Q

ALL Investigations

A
  • FBC – low haemoglobin, thrombocytopaenia and evidence of circulating leukaemic blast cells.
  • Bone marrow examination (useful for prognostic info, diagnostic)
  • Chest x-ray (mediastinal mass T-cell orientation)
    CSF for cytospin (CNS rapidly involved at first diagnosis)
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7
Q

ALL Classification

A

ALL and AML are classified by morphology. Immunological phenotyping further sub classifies ALL; the common (75%) and T-cell (15%) subtypes are the most common.

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8
Q

Signs and Symptoms of Acute leukaemia: General

A

Malaise

Anorexia

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9
Q

Signs and Symptoms of Acute leukaemia: Bone marrow infiltration

A
  • Anaemia – Pallor, lethargy
  • Neutropenia - Infection
  • Thrombocytopenia – Bruising, petechiae, nose bleeds
    Bone pain
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10
Q

Signs and Symptoms of Acute leukaemia: Reticulo-endothelial infiltration

A
  • Hepatosplenomegaly
  • Lymphadenopathy
    Superior mediastinal obstruction (uncommon)
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11
Q

Management of acute lymphoblastic leukaemia:

Remission induction –

A
  • Anaemia may require correction with blood transfusion
  • Risk of bleeding minimised by transfusion of platelets, and infection must be treated.
  • Additional hydration and allopurinol (or urate oxidase when the WCC is high and the risk is greater) – given to protect renal function against the effects of rapid cell lysis.
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12
Q

Prognostic factor high-risk features

A

Age
Tumour load (measured by the white cell count, WBC)
Cytogenic/molecular genetic abnormalities in tumour cells
Speed of response to initial chemotherapy
Minimal residual disease assessment (MRD) (submicroscopuc levels of leukaemia detected by PCR)

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13
Q

Age (high-risk)

A

10 years

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14
Q

Tumour load (measured by the white cell count, WBC)

A

> 50x109/L

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15
Q

Cytogenic/molecular genetic abnormalities in tumour cells

A

e.g. MLL rearrangement, t(4;11), hypodiploidy (

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16
Q

Speed of response to initial chemotherapy

A

Persistence of leukaemic blasts in the bone marrow

17
Q

Minimal residual disease assessment (MRD) (submicroscopuc levels of leukaemia detected by PCR)

A

high

18
Q

Outline of standard treatment

ALL

A
  1. Induction
  2. Consolidation
  3. Maintenance
  4. Intensive blocks of chemotherapy
19
Q

Induction

A

4 weeks
- Steroids (dexamethasone or prednisolone) throughout induction
- Weekly IV vincristine
- IM L-asparaginase (e.g. 9 doses in3 weeks or 2 doses of pegylated asparaginase)
- IV daunorubicin (2-4 doses, in intermediate and high risk cases)
Intrathecal (IT) methotrexate (day 18)

20
Q

Consolidation

A

CNS directed therapy
- Low risk cases: 4-wks doses of IT methotrexate and continuous oral mercaptopurine
- Higher risk cases: add IV cyclophosphamide, cytrarabine
CNS-radiotherapy only for CNS +ve cases

21
Q

Maintenance

A

Continuation treatment for at least 2 yrs (3yrs for boys)
- Daily 6-mercaptopurine (6MP), weekly oral methotrexate (doses titrated according to blood count)
- 4-weekly vincristine IV bolus and 5-day pulses of oral dexamethasone
12-weekly IT methotrexate

22
Q

Intensive blocks of chemotherapy

A

One or two blocks 8 wks duration, interrupting 1st yr of maintenance. Combinations of oral steroid, vincristine, doxorubicine, cyclophosphamide, cutarabine and L-asparaginase

23
Q

Acute myeloid Leukaemia: Aetiology

A

Accounts for 5% of all childhood malignancies and

24
Q

Acute myeloid Leukaemia: Definition

A

AML results from malignant proliferation of myeloid cell precursors.

25
Q

Acute myeloid Leukaemia: Sub-division

A

M1: AML without maturation
M2: AML with maturation
M3: acute promyelocytic leukarmia (PML)
M4: acute myelomonocytic leukaemia with eosinophilia (M4Eo)
M5: acute monocytic/monoblastic leukaemia
M6: acute erythroleukaemia
M7: acute megakaryocytic leukaemia

26
Q

Acute myeloid Leukaemia: Presentation

A
  • symptoms and signs of bone marrow replacement
  • Lymphadenopathy less prominent than in ALL
  • Intrathoracic extramedullary disease less common than in ALL
  • M3 may be present with coagulopathy from proteolytic enzyme activity
    Solid deposits (chloroma) occasionally seen in M2, M4 or M5
27
Q

Acute myeloid Leukaemia: Cytogenetics

A

Cytogenic analysis shows characteristic abnormalities:

  • M1 and M2 AML
  • M3 AML:
  • M4Eo

These translocations are regarded as good prognostic indicators. Other complex karyotypes are associated with poor risk.

28
Q

Acute myeloid Leukaemia: Prognosis

A

Overall survival is >60%

29
Q

Acute myeloid Leukaemia: Relapse AML

A

All cases require BMT after intensive re-induction, usually in conjunction with ‘FLAG’ or ‘FLAG-Ida’ regimen (i.e. fludarabine, ara-C, and G-CSF support ± idarubicin).

30
Q

Acute myeloid Leukaemia: M1 and M2 AML cytogenetics

A
  • t(8;21) translocation observed in 15% of all cases
31
Q

M3 AML: cytogenetics

A
  • t(15;17) translocation observed in 1–% of cases
32
Q

M4Eo: cytogenetics

A

inv (16) frequently observed.

33
Q

Management of AML principle:

A

Treatment – AML prolonged continuation therapy is not used:

34
Q

AML treatment

A

4 courses intensive myeloablative chemotherapy

  • The role of the gemtuzumab or myelotarg (a monoclonal antibody directed against CD33) given alongside chemotherapy is being explored in the context of clinical trials
  • PML: all-trans retinoic acid given in induction, before chemotherapy, improves survivl.
  • High risk cases, including those who fail to achieve complete remission after 2 courses, are usually offered BMT in first remission.
35
Q

Leukaemia and down syndrome:

A
  • Risk of development of acute leukaemia is 20-30times, commonly either a pre-B (common) ALL or AML (especially M7
  • Response to chemotherapy is good and better relapse-free survival is fund in those with AML
  • Patients with Down syndrome-associated leukaemia experience more complications of treatment.
36
Q

Other genetic conditions predisposing to AML:

A
  • Fanconi syndrome
  • Bloom syndrome
  • Ataxia telangiectasia
  • Kostmann’s syndrome
  • Diamond-Blackfan syndrome
  • Klinefelters
  • Turners syndrome
  • Neurofibromatosis
  • Incontinentia pigmenti

Final Notes (196 decks)

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