Childhood Leukemia Mary E. MacBlane MS, PNP-BC. Goals Incidence Etiology Diagnosis...
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Transcript of Childhood Leukemia Mary E. MacBlane MS, PNP-BC. Goals Incidence Etiology Diagnosis...
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Childhood Leukemia
Mary E. MacBlane MS, PNP-BC
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Goals
Incidence Etiology Diagnosis Types/Classification Treatment Primary Care Pearls
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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)
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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– Pesticides
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5 – Year Survival
1960 – Less than 10% Today
– ALL: 80-85%– AML: about 65%
Why? – Research – Standardized treatment protocols
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COGChildrens Oncology Group
International Organization Ongoing Studies
– Chemo combinations & timing, radiation, etc…– Quality of Life– Epidemiology
Standards of Care Treatment Protocols Nursing Discipline Shared Data Meetings
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Diagnosis: Symptoms
Fatigue Pallor Anorexia Bruising/Bleeding Fever Bone/joint pain Belly pain H/A
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Diagnosis: Exam Findings
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 Petechiae
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Differential Diagnosis
Viral Illness ITP Aplastic Anemia Arthritis Lupus Transient Erythroblastic Anemia of Childhood Other Malignancies
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Diagnostic Studies
CBC
Other– Chemistries– Uric Acid– LFT’s – LDH– Viral Titers– Chest x-ray
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CBC w/ Differential
WBC’s– ↑ or ↓
Hgb ↓ Platelet Ct ↓ Diff
– Neutropenia– Peripheral Blasts
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Types of Childhood Leukemia
ALL – Acute Lymphoblastic Leukemia AML – Acute Myeloid Leukemia CML – Chronic Myeloid Leukemia
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Classification by Cell Lineage
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Acute Lymphocytic Leukemia (ALL)
Most common cause of childhood leukemia Peak age: 2-5 years Males > females
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ALL – Best Prognosis
Ages 1-9 Females Initial WBC < 10,000 Favorable cytogenetics Early response to treatment
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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 treatment
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ALL - Cytogenetics Examples
Favorable Unfavorable
Hyperdiploid (extra chromosomes)
Hypodiploid (fewer than 54 chromosomes)
Trisomies 4, 10, 17t(9;22) BCR/ABL translocation (Philadelphia chromosome)
t (12;21) TEL-AML1 t(4;11) MLL rearrangement
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ALL - Risk Stratification
Low Risk Average (Standard) Risk High Risk Very High Risk
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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 induction
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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 males
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Acute Myeloid Leukemia (AML)
No Peak Age 20-25% of acute leukemia in children Overall prognosis about 65%
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AML – Favorable Prognosis
Down syndrome Cytogenetics: t(8;21) t(15;17), inv 16
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AML – Unfavorable Prognosis
WBC > 100,000 at diagnosis Cytogenetics: t(9;11), 11q23 Therapy-related AML Lack of remission after induction
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AML – General Treatment
Very Intensive Therapy– Induction I and II– Intensification I and II
About 6 months Inpatient for most of therapy
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Bone Marrow Transplant
High Risk ALL AML
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Complications of Leukemia Treatment
Tumor Lysis Syndrome Infection/Sepsis Thrombosis Hemorrhage / DIC Leukostasis
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Infection Risk
Central Lines Prolonged Neutropenia Immunocompromise after BMT
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Infection Risk Tidbits
Alpha Strep Fungal PCP
– Bactrim– Dapsone– Pentamidine
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Primary Care
Diagnosis During Treatment Late Effects Monitoring for relapse
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Primary Care Pearls
Diagnosis– History– Exam– CBC– No Steroids– Avoid Transfusion– CXR– Make the phone call
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Primary Care Pearls
After Diagnosis– General Care– Immunizations
Flu Shots No Live-Virus Vaccines
– Maintaining Normalcy and Hope– Sibling Considerations
Virus Varicella Flu Shots
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Primary Care Pearls
Late Effects– Avascular necrosis– Cardiotoxicity– Neuro-cognitive– Secondary malignancies– Endocrine abnormalities
Alert for Relapse
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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 blasts
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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)
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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 months
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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.
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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 deviation
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Case Study 2
High Risk T-Cell ALLAge – 12 yearsInitial WBC – 58,000Pretreated with Steroids
Already in tumor lysisCreatinine 1.1Uric acid 8.3
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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 308
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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)
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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 platelets
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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
platelets
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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
platelets
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Case Study 3
Now doing GREAT! Returned to school Visits the clinic about every 6 weeks currently
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Thank You