Lecture 9 - Heme/Onc Flashcards

1
Q

What is the second leading cause of childhood mortality?

A

cancer

trauma number 1? IDK

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2
Q

Which tumors are leading cause of cancer death in children?

A

Brain

CNS

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3
Q

Acute Leukemia

A

MC cancer

ALL (acute lymphoblastic leukemia) -75%

AML (acute myeloid leukemia) - 20%

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4
Q

ALL vs AML

A

ALL (MC)

  • M > F
  • hispanic > non-hispanic
  • caucasian > AA
  • peak incidence 2-4 years

AML
- peak incidence <2 years

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5
Q

What disorders are associated with increased risk of leukemia?

A

immunodeficiency
DNA damage/repair syndromes (Fanconi Anemia)
Down’s Syndrome

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6
Q

What are common symptoms of leukemia?

A
Weight loss 
Fever 
Frequent infections 
SOB
Weakness/fatigue 
Loss of appetite
Bone pain 
Splenomegaly 
Night sweats
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7
Q

What is the work up for possible leukemia?

A

CBC with diff
peripheral smear
tumor lysis labs
Chest Xray (+/- effusions, mediastinal mass)
Bone marrow biopsy (r/o mono or aplastic anemia)

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8
Q

What is the treatment of ALL?

A

chemotherapy induction
mostly outpt after induction
5% need bone marrow transplant

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9
Q

What is the treatment of AML?

A

6 months, inpt
more intensive and toxic than ALL tx
30% need bone marrow transplant

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10
Q

When is radiation therapy used in leukemia?

A

When CNS + diseases or T-Cell ALL

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11
Q

While on treatment for ALL or AML, if your patient relapses, what is the treatment?

A

Bone marrow transplant

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12
Q

What is the treatment for ALL or AML relapse?

A

ALL - chemo or BMT

AML - BMT

if 2+ relapse –> experimental therapy

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13
Q

Cure with Quality

A

the future goal of leukemia treatment

more individualized to avoid over or under treatment

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14
Q

Pediatric lymphoma incidence

A

10% of US childhood cancer

Non-Hodgkin’s Lymphoma - 60%
Hodgkin’s Disease - 40%

incidence increases with age - large contribution of HD in adolescence

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15
Q

Reed Sternberg

A

owl cells seen with Hodgkins disease

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16
Q

How does lymphadenopathy different between HD and NHL?

A

HD is more medial - mediastinum

NHL is more distal - axillary, groin

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17
Q

What are the differences between HD and NHL in regards to speed of spread?

A

HD: more slow

NHL: more often rapidly evolving

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18
Q

What is the Ddx for lymphadenopathy?

A
infection 
autoimmune
storage disorder
medications
vaccinations
malignancy 
histiocytosis
immunodeficiency
sarcoidosis
kawasakai 
cat-scratch
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19
Q

What is the second most common childhood malignancy?

A

brain tumors

leading cause of cancer death

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20
Q

What are the signs and sxs of brain tumor?

A
HA (MC - 1/3) 
N/V
Visual field defect 
Endocrine dysfunction (precocious or delayed puberty) 
Seizure 
Gait (intratertorial)
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21
Q

Which imaging modality is best for CNS tumor?

A

MRI

CT if rapid eval for hydrocephalous or hemorrhage

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22
Q

What are poor prognostic factors associated with CNS tumors?

A

Extent of disease (mets?)
Infancy
Low surgical accessibility

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23
Q

What is the most common solid tumor outside the CNS in children?

A

neuroblastoma (abdominal tumor)

50% dx before age 1
90% dx before age 5

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24
Q

How do sxs differ between children <1 and >1 for neuroblastoma?

A

<1 y/o

  • above the diaphragm
  • localized
  • higher prognosis

> 1 y/o

  • abdomen
  • 2/3 have disseminated dz
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25
What do you expect to see on Xray for neuroblastoma?
stippled calcifications | bone mets -irregular and lytic
26
How do you stage neuroblastomas?
CT extent of primary tumor lymph node mets
27
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
28
What is the treatment for neuroblastomas?
low risk - surgery intermediate risk - surgery + chemo high risk - surgery + chemo + irradiation + autologous BMT
29
Wilms Tumor
renal tumor 75% ages 1 -5 years 10% bilateral at dx AA > Caucasian > Asian
30
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
31
When a pt comes in with an asymptomatic abdominal mass, what must you do on PE?
A GENTLE abdominal exam ---Wilms tumors can rupture
32
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 ```
33
Osteosarcoma
MC primary bone malignancy in childhood age 12-18 years M > F metaphyses of long bones primarily affected
34
Where does osteosarcoma most commonly affect?
metaphyses of long bones 40% distal femur
35
How do pts with osteosarcoma present?
pain over involved area systemic sxs usually absent elevated Alk Phos +/- LDH
36
What is required for dx of osteosarcoma?
biopsy | performed at pediatric oncology center
37
What is the treatment for Osteosarcoma?
``` chemotherapy no radiation (tumors are radioresistant) ``` surgery - amputation or limb sparing
38
Retinoblastoma
75% dx before age 2 can be heritable (typically bilateral) or non-heritable disease Germline mutation of deletion of RB1 tumor suppressor gene
39
What are the sxs of retinoblastoma?
leukocoria strabismus proptosis neurologic sxs
40
How do you dx retinoblastoma?
ophthalmologic exam under general anesthesia
41
If the retinoblastoma is going to metastasis, where will it go?
optic nerve CNS pituitary
42
What is the treatment of retinoblastoma?
chemo local radiotherapy enucleation (eye removal)
43
What does the reticulocyte count tell you?
directly - rate of RBC production indirectly - rate of RBC destruction
44
MCV
Mean Cell (Corpuscular) Volume --average volume (size) of the RBCs
45
What are causes of decreased retic count?
Fe deficiency Lead poisoning Inflammation Bone marrow failure
46
What are causes of increased retic count?
Hemoglobinopathies RBC Membrane defects enzyme deficiencies
47
What is a normal MCV for a child >1 year?
lower limit of normal = 70 + age
48
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
49
Rhogam
Anti-Rh immunolgobulin given to all Rh negative pregnant women at 28 and 34 weeks
50
Hydrops fetalis
edema >/=2 abnormal fetal fluid collections can be caused by ABO incompatibility
51
What is the treatment, both antepartum and postpartum, for ABO incompatibility?
Antepartum: - intrauterine transfusion - induced early delivery Postpartum: -prevent kernicterus (acute bilirubin encephalopathy)
52
What are the most common microcytic anemias?
iron deficiency | thalassemia minor and major
53
What lab findings do you expect for iron deficiency?
low hemoglobin MCV, MCH, MCHC, retic, RBC count, ferritin increased TIBC, transferrin
54
What is the most likely cause of iron deficiency in a toddler?
insufficient dietary intake of iron secondary to excessive cows milk consumption
55
What are reasons why an adolescent might have iron deficiency?
Female: menstrual blood loss Occult blood loss from GI tract (Celiac disease)
56
What is the treatment for iron deficiency?
Niferex, Nu-Iron treat until lab normalize and then for an additional 2-3 months
57
What can cause increased bleeding or bruising?
``` abnormal collagen thrombocytopenia abnormal platelet function defect in VWF factor deficiency ```
58
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 ```
59
What is the most common type of hemophilia?
A - factor 8 80-85% of hemophilia cases X linked recessive
60
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 ```
61
PPC
pediatric palliative care