Page 1079 - TNFlipTest

Toronto Notes 2019
Oncology
Pediatrics P45
Investigations
• generalizedlymphadenopathy
■ CBC and differential, blood culture
■ uric acid, LDH, electrolytes
■ ANA, RF, ESR ± CRP
■ Serology: EBV, CMV and other as indicated
(e.g. HIV serology, fungal serology,
toxoplasma titre) ■ CXR
■ TBtests ■ biopsy
Leukemia
• seeHematology,H38
Epidemiology
• meanageofdiagnosis2-5yrbutcanoccuratanyage • heterogeneousgroupofdiseases
■ ALL (80%) ■ AML (15%) ■ CML (<5%)
• childrenwithDSare15xmorelikelytodevelopleukemia
Clinical Presentation
• regionallymphadenopathy
■ period of observation if asymptomatic
■ trial of oral antibiotics
■ ultrasound
■ biopsy (especially if persistent >6 wk and/or
constitutional symptoms)
Most common cause of acute bilateral cervical LAD is viral illness
• infiltrationofleukemiccellsintobonemarrowresultsinbonepainandbonemarrowfailure(anemia, neutropenia, thrombocytopenia)
• infiltrationintotissuesresultsinlymphadenopathy,hepatosplenomegaly,CNSmanifestations,testiculardisease
• fever,fatigue,weightloss,bruising,andeasybleeding
• hyperleukocytosis(totalWBC>100x109/L)isamedicalemergency
■ presents clinically with respiratory or neurological distress caused by hyperviscosity of blood and leukostasis
■ risk of ICH, pulmonary leukostasis syndrome, tumour lysis syndrome
■ management: fluids, allopurinol/rasburicase, fresh frozen plasma/platelets to correct
thrombocytopenia, induction chemotherapy, avoid transfusing RBCs unless symptomatic (and then use very small volumes)
Management
• fluids,allopurinol/rasburicase,freshfrozenplasma/plateletstocorrectthrombocytopenia,induction chemotherapy, avoid transfusing RBCs unless symptomatic (and then use very small volumes)
• combinationchemotherapyusingnon-crossresistantchemotherapyagents,allogeneicstemcell transplantation for high-grade or recurrent disease
• supportivecareandmanagementoftreatmentcomplications ■ febrile neutropenia: see Infectious Diseases, ID45
■ tumour lysis syndrome: see Hematology, H52
Prognosis
• 80-90%5yrevent-freesurvivalforALL,50-60%5-yrsurvivalforAML
• patientsarestratifiedintostandardriskandhighriskbasedonWBCandage;otherprognosticfactors
include presence of CNS/testicular disease, immunophenotype, cytogenetics, and initial response to therapy (most important prognostic variable)
Lymphoma
• seeHematology,H45
Epidemiology
• Hodgkinlymphoma:incidenceisbimodal,peaksatages15-34and>50yrold • non-Hodgkinlymphoma:incidencepeaksat7-11yr
Clinical Presentation
• Hodgkinlymphoma
■ most common presentation is persistent, painless, firm, cervical or supraclavicular
lymphadenopathy
■ can present as persistent cough or dyspnea (secondary to mediastinal mass) or less commonly as
splenomegaly, axillary, or inguinal lymphadenopathy
■ constitutional symptoms in 30% of children
■ lymph nodes become sequentially involved as disease spreads
Back pain in children must always be investigated!
Unlike adults, back pain in children often points to a pathological process