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 Caucasian gt African American ID: 1043029
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1. Childhood LeukemiaMary E. MacBlane MS, PNP-BC
2. GoalsIncidenceEtiologyDiagnosisTypes/ClassificationTreatmentPrimary Care Pearls
3. Incidence30% of all cancers in childhoodPeak incidence 2-5 years of agemales > femalesCaucasian > African AmericanIncidence of ALL (acute lymphoblastic leukemia) is 5 times higher than incidence than AML (acute myeloid leukemia)
4. EtiologyExact cause is NOT knownGeneticsIdentical twin with leukemiaChromosome abnormalitiesDown SyndromeOtherSevere Combined ImmunodeficiencyNeurofibromatosisFanconi’s AnemiaBloom SyndromeEnvironmentalIonizing RadiationChemotherapyVirusesPesticides
5. 5 – Year Survival1960 – Less than 10%TodayALL: 80-85%AML: about 65%Why? Research Standardized treatment protocols
6. COGChildrens Oncology GroupInternational OrganizationOngoing StudiesChemo combinations & timing, radiation, etc…Quality of LifeEpidemiologyStandards of CareTreatment ProtocolsNursing DisciplineShared DataMeetings
7. Diagnosis: SymptomsFatiguePallorAnorexiaBruising/BleedingFeverBone/joint painBelly painH/A
8. Diagnosis: Exam FindingsPallorBruisesPetechiaeLymphadenopathyHepatosplenomegalyCranial Nerve PalsiesTesticular enlargementChloromasLeukemia CutisMediastinal MassSuperior Vena Cava Syndrome
9. Leukemia CutisPetechiae
10. Differential DiagnosisViral IllnessITPAplastic AnemiaArthritisLupusTransient Erythroblastic Anemia of ChildhoodOther Malignancies
11. Diagnostic StudiesCBCOtherChemistriesUric AcidLFT’s LDHViral TitersChest x-ray
12. CBC w/ DifferentialWBC’s↑ or ↓Hgb ↓Platelet Ct ↓DiffNeutropeniaPeripheral Blasts
13. Types of Childhood LeukemiaALL – Acute Lymphoblastic LeukemiaAML – Acute Myeloid LeukemiaCML – Chronic Myeloid Leukemia
14. Classification by Cell Lineage
15.
16. Acute Lymphocytic Leukemia (ALL)Most common cause of childhood leukemiaPeak age: 2-5 yearsMales > females
17. ALL – Best PrognosisAges 1-9FemalesInitial WBC < 10,000Favorable cytogeneticsEarly response to treatment
18. ALL –Poor PrognosisAges < 1 year or > 10 yearsInitial WBC > 50,000Extramedullary sitesCNSTestesSteroid Pre-TreatmentUnfavorable cytogeneticsLack of remission after induction treatment
19. ALL - Cytogenetics ExamplesFavorableUnfavorableHyperdiploid (extra chromosomes)Hypodiploid (fewer than 54 chromosomes)Trisomies 4, 10, 17t(9;22) BCR/ABL translocation (Philadelphia chromosome)t (12;21) TEL-AML1t(4;11) MLL rearrangement
20. ALL - Risk StratificationLow RiskAverage (Standard) RiskHigh RiskVery High Risk
21. ALL Induction TherapyLasts 35 DaysMedicationsIntrathecal Medications weekly (Cytarabine or Methotrexate)Vincristine IV weeklyPeg-Asparaginase on Day 428 days of steroidsExamine peripheral blood for remission at Day 8 and Day 29Bone marrow recheck at Day 29Expect remission by the end of induction
22. ALL - Phases of TreatmentInduction (first month)Consolidation (1 month)Interim Maintenance I (2 months)Delayed Intensification (2 months)Interim Maintenance II (2 months)Maintenance 2 years for females3 years for males
23.
24. Acute Myeloid Leukemia (AML)No Peak Age20-25% of acute leukemia in childrenOverall prognosis about 65%
25. AML – Favorable PrognosisDown syndromeCytogenetics: t(8;21) t(15;17), inv 16
26. AML – Unfavorable PrognosisWBC > 100,000 at diagnosisCytogenetics: t(9;11), 11q23Therapy-related AMLLack of remission after induction
27. AML – General TreatmentVery Intensive TherapyInduction I and IIIntensification I and IIAbout 6 months Inpatient for most of therapy
28. Bone Marrow TransplantHigh Risk ALLAML
29. Complications of Leukemia TreatmentTumor Lysis SyndromeInfection/SepsisThrombosisHemorrhage / DICLeukostasis
30. Infection Risk Central LinesProlonged NeutropeniaImmunocompromise after BMT
31. Infection Risk TidbitsAlpha StrepFungalPCPBactrimDapsonePentamidine
32.
33. Primary Care DiagnosisDuring TreatmentLate EffectsMonitoring for relapse
34. Primary Care PearlsDiagnosisHistoryExamCBCNo SteroidsAvoid TransfusionCXRMake the phone call
35. Primary Care PearlsAfter DiagnosisGeneral CareImmunizationsFlu ShotsNo Live-Virus VaccinesMaintaining Normalcy and HopeSibling ConsiderationsVirusVaricellaFlu Shots
36. Primary Care PearlsLate EffectsAvascular necrosisCardiotoxicityNeuro-cognitiveSecondary malignanciesEndocrine abnormalitiesAlert for Relapse
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38. Case Study 12 year old female brought to primary care providerParents 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
39. Case Study 1Initial labs at admission:CBCWBC: 27,000Blasts: 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)
40. Case Study 1Treated following standard risk ALL protocol2 unplanned admissionsBoth for fever and neutropeniaOn one admission found to have pneumoniaOtherwise did well and completed therapy in 25 months
41. Case Study 212 year old male with bulky lymphadenopathy, change in voice, difficulty breathing. Seen in local ED and prescribed 4 day course of prednisoneSymptoms 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 prescribedOn 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.
42. Case Study 2Admitted to our facility with initial studies:CBC:WBC: 58.6Creatinine: 1.1 (0.5-1.2)Uric Acid: 8.3 (3.4-7.0)Chest x-ray reveals large mediastinal mass and tracheal deviation
43. Case Study 2High Risk T-Cell ALLAge – 12 yearsInitial WBC – 58,000Pretreated with SteroidsAlready in tumor lysisCreatinine 1.1Uric acid 8.3
44. Case Study 315 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 adenopathyCBCWBC 6.832% Blasts9% NeutrophilsHgb 12.6 (13-17)Plt 308
45. Case Study 3Admitted to the hospital and lumbar puncture and bone marrow completedLumbar puncture: No evidence of malignancyBone Marrow consistent with Acute Myeloid Leukemia with favorable cytogenetics: t(8,21)
46. Case Study 3Received 4 courses of chemotherapy over 4 admissionsAdmission #1 and #2 each lasted about 1 month. Received prophylactic antibiotics and antifungals. Occasional transfusions of packed cells and platelets
47. Case Study 3Admission 3: Lots of issues (1 month stay)Persistent fever despite prophylaxis. Had to change to treatment dose of meds. All cultures were negative throughout stayAmbisome for fungal coverage and then needed Amiloride to prevent potassium wastingAnorexia – Started periactin. Needed N-D tube for feedsMultiple transfusions of packed red blood cells and platelets
48. Case Study 3Admission 4: Over 6 weeks stayPersistent 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 AmilorideAnorexia – Periactin at first. Changed to Marinol. Tube feedings not tolerated. TPN required.C-diff infection. Treated with FlagylLip lesion positive for HSV-1Multiple transfusions of packed red blood cells and platelets
49. Case Study 3Now doing GREAT!Returned to schoolVisits the clinic about every 6 weeks currently
50. Thank You