Childhood Leukemia

Question 1

Malignant cell = what?

Answer 1

Blast, cells that fail to mature and crowd normal cells in the bone marrow

Question 2

What is the most common malignancy in children?

Answer 2

Leukemia, 25% of all childhood cancers

Question 3

What is the peak incidence of childhood leukemia?

Answer 3

2-5 years of age

Question 4

MC population?

Answer 4

Whites, boys

Poor prognosis in the US genetically

Question 5

ALL: age peak, % childhood leukemia, %childhood CA

Answer 5

2-4 years
75% of childhood leukemia
25% of childhood cancer

Question 6

AML age peak, % childhood leukemia, %childhood cancer

Answer 6

Age peak: neonates, teens
%Childhood leukemia: 15-20%
5% of childhood cancer

Question 7

CML age peak, %childhood leukemia, %childhood

Answer 7

Age peak: none
<5% childhood leukemia
<1% childhood CA

Question 8

Survival in childhood leukemia ALL or AML

Answer 8

ALL: 80%

AML 45%

Question 9

Acute leukemia

Answer 9

Number of leukemia cells in the bone marrow increase rapidly, death within weeks if left untreated

Question 10

Chronic leukemia

Answer 10

Insidious onset, may be asymptomatic

Death within a few years if left untreated

Question 11

Classification by cell lineage

Answer 11

Lymphoid

Myeloid: 8 subtypes of AML, much harder to treat

Question 12

ALL prognosis

Answer 12

95% in remission at end of induction, 75-85% survival at end of 2-3 years of tx

Recurrence: 50-60% chance of remission with first relapse

Question 13

AML prognosis

Answer 13

Much worse than ALL
80% remission at end of induction
50-60% relapse

Question 14

Acute leukemia clinical presentation

Answer 14

• Fatigue, pallor, anorexia
• Bruising, bleeding (low platelets)
• Fever, infection
• Bone/joint pain or refusal to bear weight/play
• Abd pain
• HA, vomiting, visual disturbances
• Tachy, petechiae/purpura
• Lymphadenopathy, HSM, CN palises, testicular involvement
• Chloromas (AML), leukemia cutis, gingivitis

Question 15

Acute leukemia symptom onset

Answer 15

-Typically 1-6 weeks prior to diagnosis

Question 16

Who looks sicker at diagnosis, AML or ALL?

Answer 16

AML

Question 17

What will you see on CBC of acute leukemia?

Answer 17

Thrombocytopenia, hgb down, neutropenia and blasts

Question 18

Chemsitry panel of acute leukemia

Answer 18

Everything up EXCEPT calcium, likely tumor lysis syndrome

Question 19

What diagnostic step is crucial in order to rule out the most important additional finding in acute leukemia?

Answer 19

CXR, must rule out mediastinal mass

Question 20

How do you obtain a definitive diagnosis of acute leukemia?

Answer 20

• Biopsy

- Get preliminary results and start tx immediately

Question 21

With cytogenetics, is hyper or hypoploidy a good prognostic factor?

Answer 21

Hyper = good
Hypo = bad

Question 22

What are you looking for on LP for diagnostic workup of acute leukemia?

Answer 22

Presence of blasts in CSF

Question 23

What is the most common subtype of ALL

Answer 23

Precursor B cell, commonly seen in preschoolers

Question 24

What subtype of ALL is commonly seen in adolescent males and is associated with mediastinal mass?

Answer 24

T cell

Question 25

What subtype of ALL responds poorly to standard ALL therapy?

Answer 25

Mature B cell

Question 26

What is the pneumonic for ALL tx and which CD is each associated with?

Answer 26

CD19 Bleotumimab CD20 Rituximab CD22 Inotuzimab CD30 Brintuximab

Question 27

Name the common immunophenotypes associateed with B lineage, T lineage, and all subtypes

Answer 27

B: 19, 22, 24, 79 T: 2, 3, 5, 7 All: CD10, TdT

Question 28

What is the absence of CD10 associated with and what is the prognosis?

Answer 28

MLL, poor prognosis

Question 29

Over what number of chromosomes is favorable?

Answer 29

Over 50, also trisomies 4, 10, and 17 favorable

Question 30

What number of chromosomes has the worst outcome?

Answer 30

Below 45, but 24-28 have the WORST

Question 31

What two associated gene products have unfavorable prognostic indications?

Answer 31

MLL and BCR/ABL (philly)

Question 32

Prognostic factors in childhood ALL (VERY HIGH RISK)

Answer 32

- BCR/ABL (philly) - Hypodiploid (<45) - Induction failure, Me by day 29

Question 33

What would lead you to believe there is remission in childhood ALL?

Answer 33

Absence of clinical signs of disease M1 marrow ANC >500 and platelets over 100k

Question 34

A patient presents with NV, HA, rash, scratchy throat, high fever, hypoTN, and tachycardia. What is going on?

Answer 34

Cytokine release syndrome, associated with high levels of IL06

Question 35

What subtype of AML is associated with down syndrome?

Answer 35

M7 (megakaryocytic)

Question 36

What 3 stains may be helpful in confirming AML diagnosis?

Answer 36

Myeloperoxidase Sudan Black B Esterase stains

Question 37

What CD are associated with AML?

Answer 37

In the middle! CD 13-15, CD 33/34, 65 HLA-DR expressed in 80% of AMLs

Question 38

Type 11 AML is what?

Answer 38

BAD, associated with MLL

Question 39

Prognostic factors in AML -- WBC, down syndrome, fab subtype, etiology

Answer 39

- Over 100k is unfavorable (more wiggle room) - Down syndrome - favorable - M3 is favorable - Treatment related etiology is unfavorable

Question 40

Potential complications of AML are similar to ALL, what is the main difference?

Answer 40

Prolonged pancytopenia

Question 41

Highlights of CML

Answer 41

Philly chromosome positive | TKI given daily