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Peds Exam 2 > Lecture 9 - Heme/Onc > Flashcards

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
Q

What do you expect to see on Xray for neuroblastoma?

A

stippled calcifications

bone mets -irregular and lytic

26
Q

How do you stage neuroblastomas?

A

CT
extent of primary tumor
lymph node mets

27
Q

How do you dx neuroblastoma?

A

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
Q

What is the treatment for neuroblastomas?

A

low risk - surgery
intermediate risk - surgery + chemo
high risk - surgery + chemo + irradiation + autologous BMT

29
Q

Wilms Tumor

A

renal tumor

75% ages 1 -5 years
10% bilateral at dx

AA > Caucasian > Asian

30
Q

How do pts with Wilms tumors typically present?

A

asymptomatic abdominal mass (70-75%)

Constipation that doesn’t resolve with treatment

X-rau with “shifting” of bowel

31
Q

When a pt comes in with an asymptomatic abdominal mass, what must you do on PE?

A

A GENTLE abdominal exam —Wilms tumors can rupture

32
Q

What is the treatment for Wilms tumor?

A 
surgical excision if possible at dx
chemotherapy 
-prevent/eradicate any mets 
-shrink tumor 
radiation therapy if local spillage or higher stage
33
Q

Osteosarcoma

A

MC primary bone malignancy in childhood
age 12-18 years
M > F

metaphyses of long bones primarily affected

34
Q

Where does osteosarcoma most commonly affect?

A

metaphyses of long bones

40% distal femur

35
Q

How do pts with osteosarcoma present?

A

pain over involved area
systemic sxs usually absent

elevated Alk Phos +/- LDH

36
Q

What is required for dx of osteosarcoma?

A

biopsy

performed at pediatric oncology center

37
Q

What is the treatment for Osteosarcoma?

A 
chemotherapy 
no radiation (tumors are radioresistant)

surgery - amputation or limb sparing

38
Q

Retinoblastoma

A

75% dx before age 2

can be heritable (typically bilateral) or non-heritable disease

Germline mutation of deletion of RB1 tumor suppressor gene

39
Q

What are the sxs of retinoblastoma?

A

leukocoria
strabismus
proptosis
neurologic sxs

40
Q

How do you dx retinoblastoma?

A

ophthalmologic exam under general anesthesia

41
Q

If the retinoblastoma is going to metastasis, where will it go?

A

optic nerve
CNS
pituitary

42
Q

What is the treatment of retinoblastoma?

A

chemo
local radiotherapy
enucleation (eye removal)

43
Q

What does the reticulocyte count tell you?

A

directly - rate of RBC production

indirectly - rate of RBC destruction

44
Q

MCV

A

Mean Cell (Corpuscular) Volume –average volume (size) of the RBCs

45
Q

What are causes of decreased retic count?

A

Fe deficiency
Lead poisoning
Inflammation
Bone marrow failure

46
Q

What are causes of increased retic count?

A

Hemoglobinopathies
RBC Membrane defects
enzyme deficiencies

47
Q

What is a normal MCV for a child >1 year?

A

lower limit of normal = 70 + age

48
Q

Anti-Rhesus Disease

A

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
Q

Rhogam

A

Anti-Rh immunolgobulin given to all Rh negative pregnant women at 28 and 34 weeks

50
Q

Hydrops fetalis

A

edema
>/=2 abnormal fetal fluid collections
can be caused by ABO incompatibility

51
Q

What is the treatment, both antepartum and postpartum, for ABO incompatibility?

A

Antepartum:

  • intrauterine transfusion
  • induced early delivery

Postpartum:
-prevent kernicterus (acute bilirubin encephalopathy)

52
Q

What are the most common microcytic anemias?

A

iron deficiency

thalassemia minor and major

53
Q

What lab findings do you expect for iron deficiency?

A

low hemoglobin MCV, MCH, MCHC, retic, RBC count, ferritin

increased TIBC, transferrin

54
Q

What is the most likely cause of iron deficiency in a toddler?

A

insufficient dietary intake of iron secondary to excessive cows milk consumption

55
Q

What are reasons why an adolescent might have iron deficiency?

A

Female: menstrual blood loss

Occult blood loss from GI tract (Celiac disease)

56
Q

What is the treatment for iron deficiency?

A

Niferex, Nu-Iron

treat until lab normalize and then for an additional 2-3 months

57
Q

What can cause increased bleeding or bruising?

A 
abnormal collagen
thrombocytopenia 
abnormal platelet function 
defect in VWF
factor deficiency
58
Q

Primary vs secondary hemostasis

A 
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
Q

What is the most common type of hemophilia?

A

A - factor 8
80-85% of hemophilia cases
X linked recessive

60
Q

What is the clinical presentation of hemophilia?

A 
bleeding from circumcision
prolonged bleeding from heel stick 
bleeding from intramuscualr injections 
swollen tender joints after minor injury 
multiple raised palpable bruises
61
Q

PPC

A

pediatric palliative care

Peds Exam 2 (11 decks)

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