Lecture 9 - Heme/Onc Flashcards
What is the second leading cause of childhood mortality?
cancer
trauma number 1? IDK
Which tumors are leading cause of cancer death in children?
Brain
CNS
Acute Leukemia
MC cancer
ALL (acute lymphoblastic leukemia) -75%
AML (acute myeloid leukemia) - 20%
ALL vs AML
ALL (MC)
- M > F
- hispanic > non-hispanic
- caucasian > AA
- peak incidence 2-4 years
AML
- peak incidence <2 years
What disorders are associated with increased risk of leukemia?
immunodeficiency
DNA damage/repair syndromes (Fanconi Anemia)
Down’s Syndrome
What are common symptoms of leukemia?
Weight loss Fever Frequent infections SOB Weakness/fatigue Loss of appetite Bone pain Splenomegaly Night sweats
What is the work up for possible leukemia?
CBC with diff
peripheral smear
tumor lysis labs
Chest Xray (+/- effusions, mediastinal mass)
Bone marrow biopsy (r/o mono or aplastic anemia)
What is the treatment of ALL?
chemotherapy induction
mostly outpt after induction
5% need bone marrow transplant
What is the treatment of AML?
6 months, inpt
more intensive and toxic than ALL tx
30% need bone marrow transplant
When is radiation therapy used in leukemia?
When CNS + diseases or T-Cell ALL
While on treatment for ALL or AML, if your patient relapses, what is the treatment?
Bone marrow transplant
What is the treatment for ALL or AML relapse?
ALL - chemo or BMT
AML - BMT
if 2+ relapse –> experimental therapy
Cure with Quality
the future goal of leukemia treatment
more individualized to avoid over or under treatment
Pediatric lymphoma incidence
10% of US childhood cancer
Non-Hodgkin’s Lymphoma - 60%
Hodgkin’s Disease - 40%
incidence increases with age - large contribution of HD in adolescence
Reed Sternberg
owl cells seen with Hodgkins disease
How does lymphadenopathy different between HD and NHL?
HD is more medial - mediastinum
NHL is more distal - axillary, groin
What are the differences between HD and NHL in regards to speed of spread?
HD: more slow
NHL: more often rapidly evolving
What is the Ddx for lymphadenopathy?
infection autoimmune storage disorder medications vaccinations malignancy histiocytosis immunodeficiency sarcoidosis kawasakai cat-scratch
What is the second most common childhood malignancy?
brain tumors
leading cause of cancer death
What are the signs and sxs of brain tumor?
HA (MC - 1/3) N/V Visual field defect Endocrine dysfunction (precocious or delayed puberty) Seizure Gait (intratertorial)
Which imaging modality is best for CNS tumor?
MRI
CT if rapid eval for hydrocephalous or hemorrhage
What are poor prognostic factors associated with CNS tumors?
Extent of disease (mets?)
Infancy
Low surgical accessibility
What is the most common solid tumor outside the CNS in children?
neuroblastoma (abdominal tumor)
50% dx before age 1
90% dx before age 5
How do sxs differ between children <1 and >1 for neuroblastoma?
<1 y/o
- above the diaphragm
- localized
- higher prognosis
> 1 y/o
- abdomen
- 2/3 have disseminated dz
What do you expect to see on Xray for neuroblastoma?
stippled calcifications
bone mets -irregular and lytic
How do you stage neuroblastomas?
CT
extent of primary tumor
lymph node mets
How do you dx neuroblastoma?
clinical suspicion
biopsy - tumor histopathology
Xray
CT staging
MRI - determines spinal cord involvement
Urine catecholamines (elevated in 90% of pts)
Bone marrow Aspirate/Biopsy
What is the treatment for neuroblastomas?
low risk - surgery
intermediate risk - surgery + chemo
high risk - surgery + chemo + irradiation + autologous BMT
Wilms Tumor
renal tumor
75% ages 1 -5 years
10% bilateral at dx
AA > Caucasian > Asian
How do pts with Wilms tumors typically present?
asymptomatic abdominal mass (70-75%)
Constipation that doesn’t resolve with treatment
X-rau with “shifting” of bowel
When a pt comes in with an asymptomatic abdominal mass, what must you do on PE?
A GENTLE abdominal exam —Wilms tumors can rupture
What is the treatment for Wilms tumor?
surgical excision if possible at dx chemotherapy -prevent/eradicate any mets -shrink tumor radiation therapy if local spillage or higher stage
Osteosarcoma
MC primary bone malignancy in childhood
age 12-18 years
M > F
metaphyses of long bones primarily affected
Where does osteosarcoma most commonly affect?
metaphyses of long bones
40% distal femur
How do pts with osteosarcoma present?
pain over involved area
systemic sxs usually absent
elevated Alk Phos +/- LDH
What is required for dx of osteosarcoma?
biopsy
performed at pediatric oncology center
What is the treatment for Osteosarcoma?
chemotherapy no radiation (tumors are radioresistant)
surgery - amputation or limb sparing
Retinoblastoma
75% dx before age 2
can be heritable (typically bilateral) or non-heritable disease
Germline mutation of deletion of RB1 tumor suppressor gene
What are the sxs of retinoblastoma?
leukocoria
strabismus
proptosis
neurologic sxs
How do you dx retinoblastoma?
ophthalmologic exam under general anesthesia
If the retinoblastoma is going to metastasis, where will it go?
optic nerve
CNS
pituitary
What is the treatment of retinoblastoma?
chemo
local radiotherapy
enucleation (eye removal)
What does the reticulocyte count tell you?
directly - rate of RBC production
indirectly - rate of RBC destruction
MCV
Mean Cell (Corpuscular) Volume –average volume (size) of the RBCs
What are causes of decreased retic count?
Fe deficiency
Lead poisoning
Inflammation
Bone marrow failure
What are causes of increased retic count?
Hemoglobinopathies
RBC Membrane defects
enzyme deficiencies
What is a normal MCV for a child >1 year?
lower limit of normal = 70 + age
Anti-Rhesus Disease
alloimmune disorder resulting in maternal sensitization and subsequent immune mediated hemolytic anemia in the fetus/neonate
erythroblasts is caused by coating the fetal RBCs with maternal IgG leading to destruction
anemia stimulates fetal erythropoesis
Rhogam
Anti-Rh immunolgobulin given to all Rh negative pregnant women at 28 and 34 weeks
Hydrops fetalis
edema
>/=2 abnormal fetal fluid collections
can be caused by ABO incompatibility
What is the treatment, both antepartum and postpartum, for ABO incompatibility?
Antepartum:
- intrauterine transfusion
- induced early delivery
Postpartum:
-prevent kernicterus (acute bilirubin encephalopathy)
What are the most common microcytic anemias?
iron deficiency
thalassemia minor and major
What lab findings do you expect for iron deficiency?
low hemoglobin MCV, MCH, MCHC, retic, RBC count, ferritin
increased TIBC, transferrin
What is the most likely cause of iron deficiency in a toddler?
insufficient dietary intake of iron secondary to excessive cows milk consumption
What are reasons why an adolescent might have iron deficiency?
Female: menstrual blood loss
Occult blood loss from GI tract (Celiac disease)
What is the treatment for iron deficiency?
Niferex, Nu-Iron
treat until lab normalize and then for an additional 2-3 months
What can cause increased bleeding or bruising?
abnormal collagen thrombocytopenia abnormal platelet function defect in VWF factor deficiency
Primary vs secondary hemostasis
Primary: Mucous membrane bleeding epistaxis prolonged oozing from minor wounds bruising, superficial ecchymoses menorrhagia abnormal intraoperative bleeding
Secondary: bleeding from large vessels subcutaneous hematomas hemarthroses intramuscular hematomas
What is the most common type of hemophilia?
A - factor 8
80-85% of hemophilia cases
X linked recessive
What is the clinical presentation of hemophilia?
bleeding from circumcision prolonged bleeding from heel stick bleeding from intramuscualr injections swollen tender joints after minor injury multiple raised palpable bruises
PPC
pediatric palliative care
