Childhood Leukemia
Mary E. MacBlane MS, PNP-BC
Goals
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Incidence
Etiology
Diagnosis
Types/Classification
Treatment
Primary Care Pearls
Incidence
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30% of all cancers in childhood
Peak incidence 2-5 years of age
males > females
Caucasian > African American
Incidence of ALL (acute lymphoblastic
leukemia) is 5 times higher than incidence
than AML (acute myeloid leukemia)
Etiology
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Exact cause is NOT known
Genetics
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Identical twin with leukemia
Chromosome abnormalities
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Other
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Down Syndrome
Severe Combined Immunodeficiency
Neurofibromatosis
Fanconi’s Anemia
Bloom Syndrome
Environmental
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Ionizing Radiation
Chemotherapy
Viruses
Pesticides
5 – Year Survival
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1960 – Less than 10%
Today
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ALL: 80-85%
AML: about 65%
Why?
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Research
Standardized treatment protocols
COG
Childrens Oncology Group
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International Organization
Ongoing Studies
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Chemo combinations & timing, radiation, etc…
Quality of Life
Epidemiology
Standards of Care
Treatment Protocols
Nursing Discipline
Shared Data
Meetings
Diagnosis: Symptoms
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Fatigue
Pallor
Anorexia
Bruising/Bleeding
Fever
Bone/joint pain
Belly pain
H/A
Diagnosis: Exam Findings
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Pallor
Bruises
Petechiae
Lymphadenopathy
Hepatosplenomegaly
Cranial Nerve Palsies
Testicular enlargement
Chloromas
Leukemia Cutis
Mediastinal Mass
Superior Vena Cava Syndrome
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Leukemia Cutis
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Petechiae
Differential Diagnosis
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Viral Illness
ITP
Aplastic Anemia
Arthritis
Lupus
Transient Erythroblastic Anemia of Childhood
Other Malignancies
Diagnostic Studies
 CBC
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Other
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Chemistries
Uric Acid
LFT’s
LDH
Viral Titers
Chest x-ray
CBC w/ Differential
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WBC’s
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↑ or ↓
Hgb ↓
Platelet Ct ↓
Diff
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Neutropenia
Peripheral Blasts
Types of Childhood Leukemia
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ALL – Acute Lymphoblastic Leukemia
AML – Acute Myeloid Leukemia
CML – Chronic Myeloid Leukemia
Classification by Cell Lineage
Acute Lymphocytic Leukemia (ALL)
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Most common cause of childhood leukemia
Peak age: 2-5 years
Males > females
ALL – Best Prognosis
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Ages 1-9
Females
Initial WBC < 10,000
Favorable cytogenetics
Early response to treatment
ALL –Poor Prognosis
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Ages < 1 year or > 10 years
Initial WBC > 50,000
Extramedullary sites
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CNS
Testes
Steroid Pre-Treatment
Unfavorable cytogenetics
Lack of remission after induction treatment
ALL - Cytogenetics Examples
Favorable
Unfavorable
Hyperdiploid (extra
chromosomes)
Hypodiploid (fewer than 54
chromosomes)
Trisomies 4, 10, 17
t(9;22) BCR/ABL
translocation (Philadelphia
chromosome)
t (12;21) TEL-AML1
t(4;11) MLL rearrangement
ALL - Risk Stratification
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Low Risk
Average (Standard) Risk
High Risk
Very High Risk
ALL Induction Therapy
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Lasts 35 Days
Medications
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Intrathecal Medications weekly (Cytarabine or
Methotrexate)
Vincristine IV weekly
Peg-Asparaginase on Day 4
28 days of steroids
Examine peripheral blood for remission at Day 8 and
Day 29
Bone marrow recheck at Day 29
Expect remission by the end of induction
ALL - Phases of Treatment
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Induction (first month)
Consolidation (1 month)
Interim Maintenance I (2 months)
Delayed Intensification (2 months)
Interim Maintenance II (2 months)
Maintenance
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2 years for females
3 years for males
Acute Myeloid Leukemia (AML)
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No Peak Age
20-25% of acute leukemia in children
Overall prognosis about 65%
AML – Favorable Prognosis
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Down syndrome
Cytogenetics: t(8;21) t(15;17), inv 16
AML – Unfavorable Prognosis
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WBC > 100,000 at diagnosis
Cytogenetics: t(9;11), 11q23
Therapy-related AML
Lack of remission after induction
AML – General Treatment
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Very Intensive Therapy
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Induction I and II
Intensification I and II
About 6 months
Inpatient for most of therapy
Bone Marrow Transplant
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High Risk ALL
AML
Complications of Leukemia Treatment
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Tumor Lysis Syndrome
Infection/Sepsis
Thrombosis
Hemorrhage / DIC
Leukostasis
Infection Risk
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Central Lines
Prolonged Neutropenia
Immunocompromise after BMT
Infection Risk Tidbits
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Alpha Strep
Fungal
PCP
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Bactrim
Dapsone
Pentamidine
Primary Care
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Diagnosis
During Treatment
Late Effects
Monitoring for relapse
Primary Care Pearls
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Diagnosis
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History
Exam
CBC
No Steroids
Avoid Transfusion
CXR
Make the phone call
Primary Care Pearls
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After Diagnosis
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General Care
Immunizations
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Flu Shots
No Live-Virus Vaccines
Maintaining Normalcy and Hope
Sibling Considerations
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Virus
Varicella
Flu Shots
Primary Care Pearls
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Late Effects
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Avascular necrosis
Cardiotoxicity
Neuro-cognitive
Secondary malignancies
Endocrine abnormalities
Alert for Relapse
Case Study 1
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2 year old female brought to primary care provider
Parents report a 1 week hx of fatique that has gotten
worse; pain that began in her feet and progressed to
legs; and a petechial rash over her arms and legs
with some bruising. She had a brief episode of
epistaxis on day prior to appointment. They also felt
that her belly has seemed more prominent for the
past 2 weeks.
Primary provider obtained a CBC which revealed
peripheral blasts
Case Study 1
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Initial labs at admission:
CBC
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WBC: 27,000
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Blasts: 34%
Neutrophils 1%
Hgb: 4.9 (11.5-13.5)
Platelets: 6,000 (150,000-400,000)
Chemistries:
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Uric Acid: 3.4 (2.4-5.7)
Potassium: 3.9 (3.3-5.1)
Creat: 0.2 (.2-.7)
Bili: 0.2 (.1-1.0)
LDH: 341 (120-300)
Case Study 1
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Treated following standard risk ALL protocol
2 unplanned admissions
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Both for fever and neutropenia
On one admission found to have pneumonia
Otherwise did well and completed therapy in
25 months
Case Study 2
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12 year old male with bulky lymphadenopathy, change in voice,
difficulty breathing.
Seen in local ED and prescribed 4 day course of prednisone
Symptoms initially resolved but recurred and seemed much
worse 3 days later (very hoarse voice, could not lay down flat to
sleep)
Again seen in ED and 5 day course of prednisone and then 4
day taper prescribed
On last day of steroids there was a biopsy of a lymph node. 2
days later the primary care provider was notified that the results
were consistent with T-cell leukemia.
Case Study 2
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Admitted to our facility with initial studies:
CBC:
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WBC: 58.6
Creatinine: 1.1 (0.5-1.2)
Uric Acid: 8.3 (3.4-7.0)
Chest x-ray reveals large mediastinal mass
and tracheal deviation
Case Study 2
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High Risk T-Cell ALL
Age – 12 years
Initial WBC – 58,000
Pretreated with Steroids
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Already in tumor lysis
Creatinine 1.1
Uric acid 8.3
Case Study 3
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15 year male who moved to the US about 6 months
earlier. He was seen in primary care office for
routine well-child check. Only complaint was
headache on and off for 2 weeks.
Exam: nl except mild submandibular adenopathy
CBC
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WBC 6.8
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32% Blasts
9% Neutrophils
Hgb 12.6 (13-17)
Plt 308
Case Study 3
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Admitted to the hospital and lumbar puncture
and bone marrow completed
Lumbar puncture: No evidence of
malignancy
Bone Marrow consistent with Acute Myeloid
Leukemia with favorable cytogenetics: t(8,21)
Case Study 3
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Received 4 courses of chemotherapy over 4
admissions
Admission #1 and #2 each lasted about 1
month. Received prophylactic antibiotics and
antifungals. Occasional transfusions of
packed cells and platelets
Case Study 3
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Admission 3: Lots of issues (1 month stay)
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Persistent fever despite prophylaxis. Had to change to
treatment dose of meds. All cultures were negative
throughout stay
Ambisome for fungal coverage and then needed Amiloride
to prevent potassium wasting
Anorexia – Started periactin. Needed N-D tube for feeds
Multiple transfusions of packed red blood cells and
platelets
Case Study 3
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Admission 4: Over 6 weeks stay
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Persistent fever despite prophylaxis. Positive blood cultures
for Staph hominis. Required Vanco and Zosyn for 10 days
and then returned to prophylaxis.
Ambisome again for fungal coverage. Required electrolyte
supplementation (Magnesium, Potassium) in addition to
Amiloride
Anorexia – Periactin at first. Changed to Marinol. Tube
feedings not tolerated. TPN required.
C-diff infection. Treated with Flagyl
Lip lesion positive for HSV-1
Multiple transfusions of packed red blood cells and
platelets
Case Study 3
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Now doing GREAT!
Returned to school
Visits the clinic about every 6 weeks currently
Thank You