Mary E MacBlane MS PNPBC Goals Incidence Etiology Diagnosis TypesClassification Treatment Primary Care Pearls Incidence 30 of all cancers in childhood Peak incidence 25 years of age males gt females ID: 488269
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Childhood Leukemia
Mary E. MacBlane MS, PNP-BCSlide2
Goals
Incidence
Etiology
Diagnosis
Types/Classification
Treatment
Primary Care PearlsSlide3
Incidence
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)Slide4
Etiology
Exact cause is NOT known
Genetics
Identical twin with leukemia
Chromosome abnormalities
Down Syndrome
Other
Severe Combined Immunodeficiency
Neurofibromatosis
Fanconi’s Anemia
Bloom Syndrome
Environmental
Ionizing Radiation
Chemotherapy
Viruses
PesticidesSlide5
5 – Year Survival
1960 – Less than 10%
Today
ALL: 80-85%
AML: about 65%
Why?
Research
Standardized treatment protocolsSlide6
COG
Childrens Oncology Group
International Organization
Ongoing Studies
Chemo combinations & timing, radiation, etc…
Quality of Life
Epidemiology
Standards of Care
Treatment Protocols
Nursing Discipline
Shared Data
MeetingsSlide7
Diagnosis: Symptoms
Fatigue
Pallor
Anorexia
Bruising/Bleeding
Fever
Bone/joint pain
Belly pain
H/ASlide8
Diagnosis: Exam Findings
Pallor
Bruises
Petechiae
Lymphadenopathy
Hepatosplenomegaly
Cranial Nerve Palsies
Testicular enlargement
Chloromas
Leukemia Cutis
Mediastinal Mass
Superior Vena Cava SyndromeSlide9
Leukemia Cutis
PetechiaeSlide10
Differential Diagnosis
Viral Illness
ITP
Aplastic Anemia
Arthritis
Lupus
Transient Erythroblastic Anemia of Childhood
Other MalignanciesSlide11
Diagnostic Studies
CBC
Other
Chemistries
Uric Acid
LFT’s
LDH
Viral Titers
Chest x-raySlide12
CBC w/ Differential
WBC’s
↑ or
↓
Hgb
↓
Platelet Ct
↓
Diff
Neutropenia
Peripheral BlastsSlide13
Types of Childhood Leukemia
ALL – Acute Lymphoblastic Leukemia
AML – Acute Myeloid Leukemia
CML – Chronic Myeloid LeukemiaSlide14
Classification by Cell LineageSlide15Slide16
Acute Lymphocytic Leukemia (ALL)
Most common cause of childhood leukemia
Peak age: 2-5 years
Males > femalesSlide17
ALL – Best Prognosis
Ages 1-9
Females
Initial WBC < 10,000
Favorable cytogenetics
Early response to treatmentSlide18
ALL –Poor Prognosis
Ages < 1 year or > 10 years
Initial WBC > 50,000
Extramedullary sites
CNS
Testes
Steroid Pre-Treatment
Unfavorable cytogenetics
Lack of remission after induction treatmentSlide19
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 rearrangementSlide20
ALL - Risk Stratification
Low Risk
Average (Standard) Risk
High Risk
Very High RiskSlide21
ALL Induction Therapy
Lasts 35 Days
Medications
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 inductionSlide22
ALL - Phases of Treatment
Induction (first month)
Consolidation (1 month)
Interim Maintenance I (2 months)
Delayed Intensification (2 months)
Interim Maintenance II (2 months)
Maintenance
2 years for females
3 years for malesSlide23Slide24
Acute Myeloid Leukemia (AML)
No Peak Age
20-25% of acute leukemia in children
Overall prognosis about 65%Slide25
AML – Favorable Prognosis
Down syndrome
Cytogenetics: t(8;21) t(15;17), inv 16Slide26
AML – Unfavorable Prognosis
WBC > 100,000 at diagnosis
Cytogenetics: t(9;11), 11q23
Therapy-related AML
Lack of remission after inductionSlide27
AML – General Treatment
Very Intensive Therapy
Induction I and II
Intensification I and II
About 6 months
Inpatient for most of therapySlide28
Bone Marrow Transplant
High Risk ALL
AMLSlide29
Complications of Leukemia Treatment
Tumor Lysis Syndrome
Infection/Sepsis
Thrombosis
Hemorrhage / DIC
LeukostasisSlide30
Infection Risk
Central Lines
Prolonged Neutropenia
Immunocompromise after BMTSlide31
Infection Risk Tidbits
Alpha Strep
Fungal
PCP
Bactrim
Dapsone
PentamidineSlide32Slide33
Primary Care
Diagnosis
During Treatment
Late Effects
Monitoring for relapseSlide34
Primary Care Pearls
Diagnosis
History
Exam
CBC
No Steroids
Avoid Transfusion
CXR
Make the phone callSlide35
Primary Care Pearls
After Diagnosis
General Care
Immunizations
Flu Shots
No Live-Virus Vaccines
Maintaining Normalcy and Hope
Sibling Considerations
Virus
Varicella
Flu ShotsSlide36
Primary Care Pearls
Late Effects
Avascular necrosis
Cardiotoxicity
Neuro-cognitive
Secondary malignancies
Endocrine abnormalities
Alert for RelapseSlide37Slide38
Case Study 1
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 blastsSlide39
Case Study 1
Initial labs at admission:
CBC
WBC: 27,000
Blasts: 34%
Neutrophils 1%
Hgb: 4.9 (11.5-13.5)
Platelets: 6,000 (150,000-400,000)
Chemistries:
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)Slide40
Case Study 1
Treated following standard risk ALL protocol
2 unplanned admissions
Both for fever and neutropenia
On one admission found to have pneumonia
Otherwise did well and completed therapy in 25 monthsSlide41
Case Study 2
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.Slide42
Case Study 2
Admitted to our facility with initial studies:
CBC:
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 deviationSlide43
Case Study 2
High Risk T-Cell ALL
Age – 12 years
Initial WBC – 58,000
Pretreated with Steroids
Already in tumor lysis
Creatinine 1.1
Uric acid 8.3Slide44
Case Study 3
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
WBC 6.8
32% Blasts
9% Neutrophils
Hgb 12.6 (13-17)
Plt 308Slide45
Case Study 3
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)Slide46
Case Study 3
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 plateletsSlide47
Case Study 3
Admission 3: Lots of issues (1 month stay)
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 plateletsSlide48
Case Study 3
Admission 4: Over 6 weeks stay
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 plateletsSlide49
Case Study 3
Now doing GREAT!
Returned to school
Visits the clinic about every 6 weeks currentlySlide50
Thank You