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