Childhood Leukemias Flashcards

1
Q

What is leukemia

A

Malignant cell - blast

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2
Q

What blasts do in leukemia

A

Baby white blood cells that fail to mature

Crowd normal cells

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3
Q

Classification of leukemia

A

Acute : sudden onset; death in weeks to months of left untreated

Chronic: insidious (gradual) onset; death in years of left untreated

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4
Q

Types of childhood leukemia lineage, onset and type

A

Lymphoid; acute; ALL
Myeloid; acute; AML
Myeloid; chronic; CML

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5
Q

Children at increased risk for leukemia

A

Down’s syndrome

Neurofibromatosis type 1

Chromosomal breakage syndromes
Fanconi anemia
Bloom syndrome
Ataxia-telangiectasia

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6
Q

Incidence of ALL and age

A

2-5 years
75-80% childhood leukemia
25% of childhood cancer

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7
Q

Incidence of AML and age

A

2-5 years
15-20% childhood leukemia
5% all childhood cancers

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8
Q

Incidence of CML and age

A

Age none
<5% childhood leukemia
<1% of all childhood cancers

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9
Q

Survival rate ALL

A

91.8%

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10
Q

Survival rate AML

A

66.4%

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11
Q

Leukemia pathophysiology

A

Genetic damage to single bone marrow white blood cell

Uncontrolled proliferation of blasts

Decreased production of normal cells

Accumulation of blasts in body
organs/ tissues ( mostly liver and spleen)

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12
Q

Acute leukemia (ALL and AML) symptom onset and clinical appearance

A

1-6 weeks prior to diagnosis (days to several months)

Children with AML often “sicker” than those with ALL at diagnosis

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13
Q

Acute leukemia clinical presentation symptoms

A

Pallor
Fatigue
Anorexia
Fever/infection
Bruising/bleeding
Bone/joint abdominal pain
Headache
Vomiting
Visual disturbances

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14
Q

Acute leukemia clinical presentation: signs

A

Pallor
Tachycardia
Petechiae/purpura
Evidence of infection

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15
Q

Acute leukemia clinical presentation: signs- organs affected (blast accumulation)

A

Lymphadenopathy (swollen lymph nodes)
Hepatosplenomegaly
Cranial nerve palsies (paralysis and involuntary tremors)
Testicular enlargement

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16
Q

Acute leukemia clinical presentation: signs-

A

Chloromas
A rare type of cancer that is made up of myeloblasts (a type of immature white blood cell) and forms outside the bone marrow and blood

Leukemia cutis
the infiltration of neoplastic leukocytes or their precursors into the epidermis, the dermis, or the subcutis, resulting in clinically identifiable cutaneous lesions.

Gingival infiltration
represents a 5% frequency as the initial presenting complication of AML

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17
Q

Diagnostic work up: CBC

A

WBC: high, low, or normal
Platelets: low
Hgb: low
Differential: neutropenia and blasts
10% of patients have normal CBC

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18
Q

Can type of leukemia be diagnosed by CBC

A

NO

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19
Q

Diagnostic work up: chemistry panel

A

Potassium, phosphorus and iron acid may be high due to (cell tumor lysis)

Calcium: low (binds with phos)

Creatinine: high due to renal insufficiency due to tumor lysis

Lactate dehydrogenase (LDH)
High. Correlates to tumor burden

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20
Q

Diagnostic work up: coagulation studies

A

Presence of coagulopathy
Indicators:
High PTT and PT
Low fibrinogen
High fibrinogen degradation products (FDP)
High d-dimer

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21
Q

FDP (fibrin degradation products)

A

Clothing and bleeding at the same time

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22
Q

Diagnostic work up additional labs

A

Viral titers:
Varicella
Hepatitis
Cytomegalovirus
Herpes simplex virus
HIV

Quantitative immunoglobulins
used to detect abnormal levels of the three major classes (IgG, IgA and IgM)

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23
Q

Diagnostic work up : chest X-ray

A

Related to mediastinal mass
T-cell leukemia subtype — THORAX
present in 5-10% of ALL
Mass may cause respiratory arrest

**always obtain chest X-ray prior to sedating patient for procedure or surgery

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24
Q

Diagnostic work up: BMA / biopsy

A

Posterior iliac crest
Marrow
Spongy bone
Cortical bone

25
Q

Diagnostic work up: bone marrow cells

A

Morphology
Immunophenotyping
Cytogenetics
Clinical features (antigens)

26
Q

Bone marrow analysis: morphology

A

Cellularity
Cell lines
Blast percentage

27
Q

Cellularity

A

Hypocellular
Hypercellular

28
Q

Cell Lines

A

Erythroid (RBC precursors)
Granulocyte (WBC precursors)
Megakaryocytic (platelet precursors)

29
Q

Bone marrow analysis: morphology
Cells classified according to

A

World Health Organization (WHO)

30
Q

Bone marrow analysis: Immunophenotyping - what does it identify

A

Markers (antigens) on blast cells

31
Q

What does Immunophenotyping help to differentiate

A

ALL vs AML
T vs B cell lineage (ALL)
Certain subtypes of AML

32
Q

What is flow cytometry

A

Using monoclonal antibodies to determine what antigens are on the leukemic cell

33
Q

Bone marrow analysis: Cytogenetics

A

How many chromosomes in leukemia cell: hyper and hypoploidy

Chromosome structure: deletions and translocations

34
Q

Diagnostic work up: lumbar puncture (LP)

A

Standard component of workup for acute leukemia

Cell count: WBC and RBC per mL of CSF

Cytology: presence or absence of blasts in CSF

IT chemo administered during diagnostic LP (usually cytarabine). Prophylactic chemo

35
Q

Went night a newly diagnosed pediatric leukemia patient have visual disturbances vomiting and a headache

A

Increased intracranial pressure from blasts

36
Q

ALL stand for

A

Acute Lymphoid Leukemia

37
Q

Lymphoid Cell Lines

A
  1. Pluripotent stem cell
  2. Lymphoid progenitor
  3. B & T lymphocytes
38
Q

Types of ALL

A

Pre-B cell (84%) - precursor B cell
T-cell ALL (15%)
Mature B cell ALL (1%) - Burkitts ALL

39
Q

Precursor B Cell ALL

A

Most common type leukemia
Commonly seen in preschoolers

40
Q

T-cell ALL

A

High WBC
Often seen with mediastinal mass
Adolescent males

41
Q

Mature b-cell ALL (burkitt)

A

Respond poorly to standard ALL therapy

Treatment same as for Burkitt lymphoma

42
Q

Cytogenetics in ALL

A

Ploidy: number of chromosomes
Diploid
Hyperdiploid
Hypodiploid

43
Q

Diploid

A

Normal 23 pairs of chromosomes for total 46

44
Q

Hyperdiploid

A

Extra copies of chromosomes
USUALLY FAVORABLE
Maybe less chemo
More common than hypo

45
Q

Hyoploidy

A

Missing copies of chromosomes
ALWAYS UNFAVORABLE
low percentile with this

46
Q

Cytogenetics : unfavorable structural change

A

Ph+ (translocation accelerator )

47
Q

What types of cancer have possible Ph+ gene

A

Mostly CML (some ALL)

48
Q

Childhood ALL: bone marrow rating

A

M1
M2
M3

49
Q

M1 bone marrow rating ALL

A

<5% blasts
GOOD!!
Means remission

50
Q

M2 bone marrow rating ALL

A

5 to <25% blasts in marrow

51
Q

M3 bone marrow rating ALL

A

> or equal to 25% blasts
BAD

52
Q

What is the most important prognostic factor in childhood ALL

A

MRD: minimal residual disease
The time it takes to eliminate the bulk of leukemia

53
Q

MRD can now be tested how instead of a microscope

A

Blood test. 100x more sensitive than microscope

54
Q

Measurement of MRD by bone barrow sample is done when

A

End of induction
3-4 months

55
Q

At end of induction what is favorable MRD

A

<.01% or undetectable

56
Q

At end of induction what is unfavorable MRD

A

Persistent MRD > or equal to 0.01% for B lineage ALL

57
Q

3-4 months out what is a favorable MRD

A

Low MRD <0.01% or undetectable

58
Q

3-4 months out what is a unfavorable MRD

A

Persistence of MRD > or equal to 0.01% for both B and T lineage ALL