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Childhood Leukemia Childhood Leukemia

Childhood Leukemia - PowerPoint Presentation

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Childhood Leukemia - PPT Presentation

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

case leukemia risk study leukemia case study risk treatment care day aml primary 000 diagnosis acute cbc wbc months

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Slide1

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 LineageSlide15
Slide16

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 malesSlide23
Slide24

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

PentamidineSlide32
Slide33

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 RelapseSlide37
Slide38

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