AML is a disease of what group?
most common in adults, though it is seen from childhood through all groups
CML is a disease of what group?
middle-age and older adults (can rarely occur in children/adolescents)
How does one stage a lymphoma/leukemia?
CT or MRI: allows ID of solid tumors in the brain, chest, abdomen, pelvis, and lymph node chains
lumbar puncture is also done at this time
In children, the primary cancers are ______________ or _______________. ___% are metastatic at the time of dx.
In adults, the primary cancers are ________ in origin. This means that identification of the neoplasia occurs _______.
earlier (only 20% have metastasis at the time of dx)
What kind of effects can pediatric oncology have on normal growth?
Radiation increase the risk of adult epithelial cancers
Childhood cancers are mostly more responsive to chemo than adult cancers, and children suffer fewer tx-related side effects than adults.
What is the incidence of ALL?
Accounts for 77% of childhood leukemias
Peak incidence is 2-6 years old, male > female
What is the etiology of ALL?
GENETIC ABNORMALITIES (Down's, Bloom, ataxia-telangectsia syndrome, Fanconi anemia)
IONIZING RADIATION the only causal relationship in U.S.
EBV is causal for B-cell ALL in developing nations
What are the symptoms of ALL?
Non-specific low-grade fever, irritability, anorexia, fatigue
BONE PAIN in lower extremities is less common in early stages
Late stages of BM failure: PALLOR, BRUISING, EPISTAXIS, RECURRENT INFECTION
What do you see on physical exam with ALL?
Pallor, petechiae/purpura, listlessness (BM failure)
tender, swollen joints
supine respiratory distress (large anterior mediastinal mass)
papilledema, cranial nerve palsies, retinal hemorrhages (signs of increased intracranial pressure)
What is papilledema?
Swelling of the optic nerve causes blurring of the optic nerve at physical exam
What is retinal hemorrhage?
Bleeding of the blood vessels in the retina, in this case due to low platelet counts
What is cranial nerve palsy?
Increased intercranial pressure causes the cranial nerves to lose partial or complete function
First to be affected is usually CNVI (abducens)
What are the lab findings in ALL?
Bone marrow aspirate: >25% cells are homogeneous population of lymphoblasts
CSF analysis to look for lymphoblasts (means additional therapies in treatment plan)
What is the treatment for ALL?
Initial treatment = INDUCTION - goal is to eradicate leukemic cells from the marrow (a.k.a. REMISSION)
~ 4 weeks
Phase 2 (CONSOLIDATION) involves lumbar puncture/chemo to prevent CNS relapse.
Patients with t(9;22) (& some others) receive bone marrow transplantation
Phase 3 (MAINTENANCE) (~2-3 years) = monitoring for relapse (15-20% of pts)
Overall survival > 80% at 5 years
What are the side effects of ALL treatment?
tumor lysis syndrome, renal failure, myelosuppression, infection (immunosuppressed, plus central line)
What can be given for tumor lysis syndrome?
allopurinol to remove uric acid
loop diuretic for calcium and phosphate
bicarb to reverse hyperkalemia
if no improvement, then hemodialysis
What is the incidence of AML?
most common adult leukemia in adults (80%), mostly male
11% of childhood leukemias
More common with genetic abnormalities (Down's, Bloom, Fanconi anemia, ataxia-telectangiectsia, others)
What is the etiology of AML?
IONIZING RADIATION, chemotherapy, organic solvents
possibly ↑ risk in smokers; genetics
What is the physical presentation of AML?
Signs of marrow failure
"Blueberry muffin" lesions - papable, purpuric subcutaneous nodules (AKA Leukemia Cutis)
What is the pathophysiology of gingival bleeding?
Myelogenous cells infiltrate the gingiva, causing swelling and bleeding
Form of cutaneous AML involvement
What are the labs for AML?
normocytic, normochromic anemia
BM: hypercellular marrow with >30% blasts
karyotyping has prognostic and tx value
What is the treatment for AML?
induction chemo achieves remission in 80%; 105 die from infection/bleeding prior to remission
SCT or BMT after remission from an appropriate donor can improve survival (graft-vs-leukemia effect favors allogeneic transplantation)
In lieu of transplant, maintenance chemo can be curative for some
Here's a review for CML.
2-3% of all childhood leukemia
Features the Philadelphia chromosome with subsequent BCR-ABL gene rearrangement
Presents with splenomegaly, thrombocytosis, leukocytosis
Chronic phase in children lasts 3-4 years, followed by blast crisis, chronic phase may last 10-15 years in adults
Treatment with imatinib (gleevec) results in 70% major response
Stem cell transplantation or bone marrow transplant results in 80% cure rate