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Pediatric Board Review COPY > Hematology-Oncology > Flashcards

Hematology-Oncology Flashcards

1
Q

What are some high risk indicators for ALL?

A
  • Age 10
  • WBC >50,000 (T-cell>B cell
  • prior steroid therapy
  • testicular disease
  • hypodiploid (<44 chromosomes)
  • Ph+
  • poor initial response
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2
Q

Findings in Tumor Lysis Syndrome

A
  • Hyperuricemia
  • Hyperphosphatemia
  • Hypocalcemia
  • Hyperkalemia

(when Ca/PO4 is >60 precipitation will occur in brain, collecting tubules in kidney etc.)

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3
Q

Hyperkalemia EKG changes?

A
  • 6 –> peaked T waves
  • 7 –> prolonged PR interval
  • 8 –> absent P wave/widened QRS
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4
Q

Features of AML

A
  • no peak age
  • no sex predilection
  • leukoerythroblastic reaction - primitive WBC, nRBC, teardrop RBC
  • M1, M2, M3 –> Auer rods
  • hyperleukocytosis common –> sludging
  • extramedullary involvement (chloromas, leukemia cutis, gum hypertrophy, CNS disease)

(nucleated RBC = 1. ineffective erythropoesis, 2. functional asplenia, 3. marrow replacement

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5
Q

High risk prognostic factors in AML?

A
  • monosomy 7
  • monosomy 5/5q
  • FLT 3 internal tandem duplication
  • > 15% blasts after first cycle of induction
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6
Q

Good prognostic factors in AML:

A
  • trisomy 8, t(8:21) higher remission rate
  • inv(16) or t(16;16) associated with M4 (eos) presentation
  • Trisomy 21 pt
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7
Q

Genetic diseases associated with an increased risk of leukemia?

A
  • Klinefelter’s
  • Bloom’s
  • Fanconi’s Anemia
  • Neurofibromatosis
  • Kostman’s Neutropenia
  • Schwachman’s

*AML is most frequent secondary malignancy in survivors of Hodgkin’s Disease

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8
Q

Mediastinal Mass

A

T-cell ALL

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9
Q

DIC

A

APML (M3); t(15;17), ATRA/As2O3

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10
Q

Extramedullary disease, infants

A

myelomonocytic/monocytic (M4/M5)

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11
Q

Pancytopenia, Down Syndrome

A

Megakaryoblastic (M7)

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12
Q

Name the cancer:

  • mean age mid-40’s
  • persistent neutrophilia without infection
  • absolute basophilia
  • thrombocytosis in chronic phase
  • low LAP score in chronic phase
  • increased serum B12
A

CML

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13
Q

Tumor Markers - Hodgkin’s

A

ESR, Copper

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14
Q

Tumor Markers - Neuroblastoma

A

Urine catecholamines, ferritin

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15
Q

Tumor Markers - Hepatoblastoma

A

Alpha fetoprotein

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16
Q

Tumor Markers - Teratocarcinoma, YST

A

Alpha fetoprotein

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17
Q

Tumor Markers - Embryonal Carcinoma, Choriocarcinoma, Seminoma

A

Beta HCG

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18
Q

Bad: hypodiploid, t(9:22)
Good: triple trisomy (4, 10, 17), TEL/AML = t(12:21)

A

ALL

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19
Q

Bad: monosomy 7
Good: Down Syndrome

A

AML

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20
Q

t(15;17)

A

APML

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21
Q

t(9;22) = BCR-abl; Philadelphia chromosome

A

CML

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22
Q

t(8;14) involves MYC oncogene

A

Burkitt’s Lymphoma

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23
Q

Good: hyperdiploid (infants)
Bad: NMYC amplification, 1p-

A

Neuroblastoma

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24
Q

11p- (sporadic cases)

A

Wilm’s Tumor

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25
Q

13q- = Rb tumor suppressor

A

Retinoblastoma

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26
Q

t(11;22) = EWS-Fli

A

Ewing’s/PNET

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27
Q

High risk factors for ALL

A
  • prior treatment with steroids
  • 12 year old patient
  • testicular involvement
  • residual leukemia after induction
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28
Q

High risk factors for AML

A
  • monosomy 7

- residual leukemia after induction

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29
Q

Associated with Wilm’s Tumor, Neuroblastoma, Both or Neither?

Abdominal Mass

A

Both

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30
Q

Associated with Wilm’s Tumor, Neuroblastoma, Both or Neither?

Peripheral blasts

A

Neuroblastoma

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31
Q

Associated with Wilm’s Tumor, Neuroblastoma, Both or Neither?

Hypertension

A

Both

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32
Q

Associated with Wilm’s Tumor, Neuroblastoma, Both or Neither?

Elevated serum ferritin

A

Neuroblastoma

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33
Q

Associated with Wilm’s Tumor, Neuroblastoma, Both or Neither?

Rapid, irregular eye movements

A

Neuroblastoma

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34
Q

Associated with Wilm’s Tumor, Neuroblastoma, Both or Neither?

Commonly metastasizes to lungs

A

Wilm’s

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35
Q

Associated with Wilm’s Tumor, Neuroblastoma, Both or Neither?

Elevated alpha fetoprotein

A

Neither

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36
Q

Associated with Wilm’s Tumor, Neuroblastoma, Both or Neither?

Most common in toddler age group

A

Both

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37
Q

Associated with osteogenic sarcoma, ewing’s sarcoma, both or neither?

more common in second decade of life

A

Both

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38
Q

Associated with osteogenic sarcoma, ewing’s sarcoma, both or neither?

Li-Fraumeni Syndrome

A

Osteogenic sarcoma

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39
Q

Associated with osteogenic sarcoma, ewing’s sarcoma, both or neither?

Onion skin appearance on x-ray

A

Ewing’s

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40
Q

Associated with osteogenic sarcoma, ewing’s sarcoma, both or neither?

Metaphyseal location

A

Osteogenic sarcoma

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41
Q

Associated with osteogenic sarcoma, ewing’s sarcoma, both or neither?

Down Syndrome

A

Neither

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42
Q

Associated with osteogenic sarcoma, ewing’s sarcoma, both or neither?

Fever

A

Ewing’s

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43
Q

Associated with osteogenic sarcoma, ewing’s sarcoma, both or neither?

Pulmonary metastasis

A

Both

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44
Q

t(9;22)

A

Poor risk ALL

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45
Q

retinoic acid

A

APML (FAB M3)

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46
Q

translocation involving c-myc

A

Burkitt’s lymphoma

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47
Q

rarely occurs in Blacks

A

Ewing’s sarcoma

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48
Q

high risk ALL

A

hypodiploid chromosomes

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49
Q

LCH

A

Birbeck granules

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50
Q

Burkitt’s lymphoma

A

Starry sky histology

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51
Q

AML

A

Auer rod

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52
Q

Hodgkin’s lymphoma

A

Reed-Sternberg cell

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53
Q

Neuroblastoma

A

Elevated ferritin

54
Q

Osteogenic sarcoma

A

Elevated alkaline phosphatase

55
Q

High WBC, blasts, DIC

A

APML (Promyelocytic) M3

56
Q

Gingival hypertrophy, CNS disease

A

Monoblastic (M5)

57
Q

Mediastinal mass

A

T-cell ALL

58
Q

Down syndrome

A

Megakaryoblastic (M7)

59
Q

Chloroma

A

Monoblastic (M5)

60
Q

Testicular relapse

A

T-cell ALL

61
Q

1p-

A

poor prognosis neuroblastoma

62
Q

Monosomy 7

A

poor prognosis AML

63
Q

11 p-

A

Wilm’s tumor

64
Q

13q-

A

Retinoblastoma

65
Q

Trisomy 21

A

good prognosis AML

66
Q

5y/o M with DI and lytic skull lesions

A

Langerhans Cell Histiocytosis

67
Q

12y/o M with pallor, new onset wheezing and cervical/supraclavicular adenopathy

A

T-cell ALL

68
Q

14y/o M with massive splenomegaly on routine PE

A

CML

69
Q

6y/o M with RLQ pain/mass

A

Burkitt’s

70
Q

16y/o boy with painless cervical and supraclavicular adenopathy

A

Hodgkin’s Lymphoma

71
Q

2y/o F with painless abdominal mass and hematuria

A

Wilm’s Tumor

72
Q

3y/o F with pallor, periorbital ecchymoses, hip pain

A

Neuroblastoma

73
Q

14y/o M with painful swelling of right mid-femur

A

Ewing’s

74
Q

5y/o M with repeated bouts of vomiting

A

Cerebellar brain tumor

75
Q

Hyperpigmentation, micro-cornea, thumb abnormalities

A

Fanconi’s Anemia

76
Q

Globin chains in HbF

A

a2g2

77
Q

Globin chains of HbA

A

a2B2

78
Q

Globin chains of HbA2

A

a2d2

79
Q

Hb H

A

alpha-thal; unstable; non-functional

80
Q

Barts (g4)

A

alpha-thal in BABIES

81
Q

When do beta chain problems (SCD, beta thal) present?

A

4-6mos of age

82
Q

Things that shift the O2 dissociation curve to the left?

A

HbF

83
Q

Things that shift the O2 dissociation curve to the right? (Incr delivery to tissues)

A

Acidosis, hypoxia, 2,2DPG

84
Q

Chocolate brown arterial blood

A

METHEMOGLOBINEMIA:

  • Fe2+ to Fe3+ (can’t bind O2)
  • exposure to nitrites, NO, pyridium
  • AGE in infants –> stool bicarb losses –> acidosis –> bacterial overgrowth –> increased nitrites
  • Rx: methylene blue
  • Don’t use in G6PD - insufficient NADPH for drug to work and may lead to hemolysis
85
Q

What is lost first - folate stores or B12 stores?

A

Folate - small store; deficiency in 1month, anemia within 4 months of deprivation

B12 - large stores; deficiency usually due to an absorptive problem - PA, ileal resection, fish tapeworm, or vegan diet

Folate will fix anemia but won’t stop neurologic deterioration from lack of B12

Look for HYPERSEGMENTED POLYS

86
Q

Normocytic anemia, low serum Fe, but increased ferritin. Low epo levels relative to degree of anemia

A

Anemia of Chronic Inflammation

87
Q

What decreases Fe absorption in the small intestine?

A

Oxalates

Vitamin C improves absorption

88
Q

12mo boy with a Hct of 27%, MCV 69. Breast fed until 6mos of age, then given whole milk.

A

Iron Deficiency

  • determine the cause
  • ferritin –> serum Fe/FEP –> anemia
  • continue Fe therapy until ferritin normal
  • peak incidence between 6mos and 3 years (times of greatest growth)
89
Q

The most important treatment for Acute Chest Syndrome?

A

Transfusion

90
Q

What finding on a peripheral blood smear is most indicative of loss of splenic function in sickle cell disease?

A

nRBC

91
Q

2y/o with 1 day h/o painful swelling over hands and feet with low grade fever.

A

SCD = dactylitis

92
Q

Target cells, think…

A

Hb SC

93
Q

A predominance of Hb Barts is associated with a defect in how many globin genes?

A

4

94
Q

A 4y/o Vietnamese M with a microcytic, hypochromic anemia, Hb of 9, no response to therapeutic trial of iron

A

Alpha-thalassemia

95
Q

3y/o Italian-American boy with mild microcytic, hypochromic anemia and hepatosplenomegaly. Hb 11.3, MCV 61, RBC 5 x 10^6. Smear shows target cells and basophilic stippling.

A

Beta-Thalassemia

96
Q

Hair on end XR?

A

Beta thalassemia

97
Q

Heredity of G6PD

A

X-linked

98
Q

5m/o with recurrent skin infections and hypopigmentation of skin, hair and eyes. CBC shows moderate neutropenia with giant neutrophil granules

A

Chediak-Higashi

99
Q

Giant neutrophil granules

A

Chediak-Higashi

100
Q

Hb Bart’s

A

Alpha-thalassemia

101
Q

NBT test

A

CGD

102
Q

Hydrops fetalis

A

Alpha-thal

103
Q

Infections with catalase positive bacteria

A

CGD

104
Q

Hb F

A

Higher O2 infinity

105
Q

Which thal?

In its MOST SEVERE FORM is characterized by both ineffective erythropoiesis AND hemolytic anemia

A

both

106
Q

Which thal?

The severity of clinical presentation is proportional to dose of defective genes

A

alpha thal

107
Q

Which thal?

Hb H is present

A

alpha

108
Q

Which thal?

Elevations of Hb A2 and Hb F

A

beta

109
Q

Which thal?

Diagnosis often made in newborn period

A

Alpha

110
Q

Which thal?

Iron responsive microcytic anemia

A

Neither

111
Q

Which thal?

Errors in transcription or translation of globin mRNA

A

beta

112
Q

Folate or B12?

Hypersegmented polys

A

both

113
Q

Folate or B12?

Poor diet, pregnancy, presence of chronic hemolysis

A

Folate

114
Q

Folate or B12?

Ileal resection

A

B12

115
Q

Folate or B12?

Normochromic/normocytic morphology, low retic

A

Neither

116
Q

Folate or B12?

Dyphyllobothrium latum infestation

A

B12

117
Q

Folate or B12?

Exclusively breast fed infants of strict vegan mothers

A

B12

118
Q

Fe deficiency or lead poisoning?

Hypochromic/microcytic anemia

A

both

119
Q

Fe deficiency or lead poisoning?

Acute encephalopathy

A

Lead poisoning

120
Q

Fe deficiency or lead poisoning?

Elevated Free Erythrocyte Protoporphyrin (FEP)

A

Both

121
Q

Fe deficiency or lead poisoning?

More common in children of low income families

A

both

122
Q

Dimercaprol (BAL)

A

Lead poisoning

123
Q

Fe decreased but ferritin increased

A

Chronic inflammation

124
Q

B4 globin tetramer

A

Hb H

125
Q

Parvovirus

A

Aplastic Crisis

126
Q

Low EPO level

A

Chronic inflammation

127
Q

rIFN-gamma

A

CGD

128
Q

Malaria belt

A

G6PD

129
Q

Non-megaloblastic macrocytosis

A

Liver disease

130
Q

An 18m/o F is brought in for looking pale. PMH is unremarkable. There is a h/o URI 1 week prior. She eats a regular diet and is alert and active. Except for pallor PE is normal. CBC shows normal WBC and plt count but a Hb of 4.5 with MCV of 74. The most likely diagnosis is…

A

TEC

131
Q

A 2m/o F is brought in for looking pale. CBC shows normal WBC and PLT count but a Hb of 5.5 with MCV of 105. The most likely diagnosis is…

A

Diamond Blackfan Anemia

132
Q

A 3y/o F is well below 5th percentile for height and weight. Her PMx is unremarkable. Her PE shows micrognathia, hypoplastic thumbs and areas of hyperpigmentation on her chest and thighs. Her CBC is normal. The most likely hematologic diagnosis is…

A

Fanconi Anemia (DEB chromosome breakage study is test of choice)

Pediatric Board Review COPY (17 decks)

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