Download Pediatric Hemato

Pediatric Hemato-Oncology
Hee Young Shin
Seoul National University
Children’s Hospital
Pediatric Cancer in Korea
• 130/per million population
• Population under the age 15: Among 48 million
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20% 9.60 million
Around 1,200 new cases per year
Acute Leukemeia
30 -35%
Brain tumor
15%
Malignant Lymphoma
10%
Solid tumors: Neuroblastoma,
rhabdomyosarcoma, Wilms tumor
Survival of childhood cancers
• Average:
• ALL
• AML
over 70%
over 80%
60 ~ 70%
– Sibling transplant
70%
– Unrelated BMT
60%
– Cord blood transplant 50%
– Autologous PBSCT
70%
Survival of childhood cancers
• Brain tumor 60 %
– Medulloblastoma 60 ~ 70%
– Germ cell tumor 80 ~ 90%
– Glioma: brain stem glioma 0%
• Malignant Lymphoma
– NHL 80%
– Hodgkin 80%
Survival of childhood cancers
• Neuroblastoma
– Stage 1, 2: 90%
– Stage 3, 4: variable to treatment
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Wilms tumor: 90%
Rhabdomyosarcoma: > 80%
Osteosarcoma: > 80%
Germ cell tumor: > 80%
Hepatoblastoma: > 80%
Characteristics of childhood cancers
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Origin of tumor: mesodermal
Rapid growth: response to chemo
Survival
Growing child: regeneration
Growth and development: school
Social adaptation
Total care, total healing environment
Etiology of childhood cancers
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Factors: radiation, chemicals etc
Worldwide incidence: IARC
130/million
Lottery
Parents: guilty feeing - projection
Acute Leukemia
• ALL: AML ratio
• Principle of treatment
• Diagnosis of ALL/AML
– Morphology: size, N/C ratio, nucleoli
– Special staining: PAS, SBB, peroxidase
– Immunologic marker: FACS
ALL: Prognostic factors
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Age at diagnosis
WBC at diagnosis
FAB classification
Immunologic marker
Chromosome: Ph(+), translocation,
hypodiploidy
• Day 7 BM: M1, M2, M3
ALL: Treatment Principle
• Phase of treatment
– Induction: 3 drug/ 4 drug
– Consolidation: IT / RT
– Interim maintenance (1st, 2nd)
– Delayed intensification (1st, 2nd)
• Reinduction
• Reconsolidation :I
– Maintenance: M 3yr, F 2yr from 1st IM
ALL: Complication of therapy
• Acute tumorlysis syndrome
– Uric acid nephropathy
– Hyperkalemia
– Hyperphosphatemia/hypocalcemia
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DM: PD, L-asp
Neutropenia – infection
Pancreatitis: L-asp
Thromboembolism: L-asp
Muscular atrophy: exercise
Hyperleukocytosis
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Leukostasis: ischemia – infarct
Hydration
RT
Leukapheresis
RBC transfusion
Plt transfusion
AML
• Induction: 3&5, 3&7, Denver, BH-AC
• Consolidation
• Stem cell transplantation
– Sibling related
– Unrelated
– Umbilical cord blood
– Auto PBSCT
Stem cell transplantation
• Conditioning
– Marrow ablation
– Immune system
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Stem cell infusion
Aplasia: Laminar flow room
Engraftment
GVHD/GVL: cyclosporin
CMV infection
CML
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Philadelphia chromosome
Glivec
Low dose Ara-C
Interferon
Stem cell transplantation
JMML
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Philadelphia chromosome (-)
Skin lesion
Fever
Splenomegaly
Stem cell transplant
Neutropenia with fever
• Inflammation – WBC
• Criteria of infection
– Cough: pneumonia
– Tenderness: cellulitis
– Otalgia: AOM
– Dysuria: UTI
• Timing of antibiotics: most important
• Prevention: gargle, sitz bath
Brain tumor
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Medulloblastoma/PNET
Blood-Brain barrier
8-in-1 chemotherapy
Surgery
Radiation therapy
Germ cell tumor
Glioma
NHL
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Systemic disease
High grade
Induction/Maintenance
Burkitt
– Intensive, short-term treatment
– Tumorlysis syndrome
Neuroblastoma
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Stage
N-myc amplification
Diagnosis: biopsy
Treatment: PBSCT
IL-2
ITT
Rhabdomyosarcoma
• Site: pelvis, head and neck
• Pathology: alveolar
• IRS-III, IV
• POG ICE
Wilms tumor
• Associated anomaly
• Diagnosis
• Treatment sequence
– NWTS
– SIOP
• Hepatopathy-thrombocytopenia synd
– OT/PT, thrombocytopenia
Osteosarcoma
• Initial W/U
• Choice of treatment
– Intraarterial
– IV chemotherapy
• Prognosis
• Complication
– Renal failure
– Pulmonary fibrosis
– Cardiac failure
• Recurrence: primary site, lung
Hepatoblastoma
• Diagnosis: normal level of α-FP
• Prognositic factor: resectability
• DDx:
– Hemangioendothelioma
– Teratoma
– Hepatocelluar carcinoma
Tumor markers in pediatric HO
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α-FP
β-hCG
NSE
VMA/Cr
Diagnosis
Response of treatment
Relapse
Retinoblastoma
• Hereditary: 40%
• Rb gene
• Indication of chemotherapy
• Role of PBSCT
Immune function after chemotherapy
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Cell mediated immune function
Humoral immune function
IMIG
Herpes zoster
Vaccination after off-therapy