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PEDS > Hematology/ ONcology > Flashcards

Hematology/ ONcology Flashcards

1
Q

3 possibilities for causes of anemia

A

reduced production
increased destruction
loss of RBCs

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2
Q

in what type of anemia will MCV be low?

A

iron deficiency

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3
Q

what type anemia is due to red cell aplasia?

A

Diamond Blackfan anemia

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4
Q

is “normal” reticulocyte count in the face of anemia normal?

A

No, it is abnormal

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5
Q

how to you tx diamond blackfan anemia?

A

steroids

BMT is curative

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6
Q

if reticulocyte count is increased and there is anemia what is happening?

A

hemolysis

blood loss

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7
Q

what are intrinsic problems that cause hemolysis leading to anemia?

A

membrane defects- hereditary spherocytosis
enzyme-G 6PD deficiency
Hemoglobinopathies

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8
Q

what is the most common childhood hematologic problem?

A

anemia

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9
Q

what does jaundice with anemia indicate?

A

hemolysis

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10
Q

where do all RBCs go to die?

A

spleen

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11
Q

presents with Pallor, fatigue, dizziness, poor growth, exercise intolerance
Tachycardia, murmur, headache, syncope

A

anemia

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12
Q

anemia w/ lymphadenopthy can indicate what?

A

leukemia
HIV
JRA

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13
Q

anemia with splenomegaly can indicate what?

A

leukemia
sickle cell
hemoglobinopathy
spherocytosis

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14
Q

what are the three cell lines?

A

red cells
white cells
platelets

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15
Q

when 2 cell lines are depressed what is there a higher likelihood for?

A

leukemia

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16
Q

what do schistocytes indicate?

A

HUS

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17
Q

what can blister cells indicate?

A

G6PD

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18
Q

should iron deficiency show up early in life?

A

no, have iron stores from mom for first 5-6 months of life

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19
Q

what causes iron deficiency anemia in kids 6-24 months

A

poor dietary intake (ie cows milk)

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20
Q

tx for iron deficiency anemia

A

6 mg/ kg/ day divided TID

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21
Q

why does cows milk lead to iron deficiency anemia?

A

calcium in cows milk binds the iron

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22
Q

what are 2 macrocytic anemias?

A

Folate and B 12 deficiency

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23
Q

what does irritable, weakness, paresthesias, unsteady gait indicated deficiency in

A

Vit B 12

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24
Q

congenital aplastic anemia. Will have congenital anaomlaies, thrombocytopenia. diagnosed in 2-10 year olds

A

fanconi’s

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25
Q

just affects red cell lines. shows up at birth- 1 year.

A

diamond black-fan

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26
Q

what are 2 congenital anemias

A

fanconi

diamond black fan

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27
Q

what can cause acquired anemias

A

idiosyncratic rxn to meds
after an infection
idiopathic (mostly)

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28
Q

in acquired anemias are there HSN or jaundice?

A

No

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29
Q

what causes isoimmunization hemolysis?

A

Rh/ABO/ minor blood groups

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30
Q

what are congenital hemolysis?

A

sphereocytosis
G6PD deficiency
pyruvate kinase deficiency
hemoglobinopathies

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31
Q

autosomal dominant. Managed with splenectomy. cells look like spheres.

A

Hereditary spherocytosis

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32
Q

why do you take the spleen out with hereditary spherocytosis

A

so the spleen stops killing the red blood cells

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33
Q

Most frequent inherited enzyme defect
X-linked recessive (males)
Anemia, hyperbilirubinemia* jaundice
Episodic hemolysis with oxidative stress

A

G6PD deficiency

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34
Q

what is the 2nd most commonly inherited enzyme defect

A

pyruvate kinase deficiency

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35
Q

when do symptoms of beta thalassemias show up?

A

4 months due to fetal hemoglobin

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36
Q

when do symptoms of sickle cell onset?

A

3-4 months

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37
Q

what will you have with sickle cell dz?

A

splenomegaly due to sequestration

recurrent episodes of vasoocclusion and tissue ischemia

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38
Q

what should be given for prophylaxis for sickle cell dz?

A

penicillin (pneumococcal, meningococcal, H. flu)

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39
Q

what are 3 general categories of platelet disorders

A

Decreased production
Increased consumption
Impaired function

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40
Q

what are 3 disorders of coagulation

A

Hemophilias
Vitamin K deficiency
DIC

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41
Q

what measure the intrinsic pathway?

A

aPTT

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42
Q

what measures the extrinsic pathway?

A

PT

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43
Q

kids show up with a lot of petechiae and purpura what do you suspect?

A

ITP

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44
Q

what causes decreased production leading to thrombocytopenia

A 
infiltrative diseases (affect bone marrow)
congenital production defects
viral infection?
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45
Q

large vascular venous malformation

A

Kasabach-Merritt syndrome

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46
Q

what cause increased consumption leading to thrombocytopenia (more common)

A

ITP
DIC
Kassabach-Merrit syndrome

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47
Q

what causes thrombocytopenia by impaired function

A 
Von Wilebrand dz
class test- bleeding time
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48
Q

in peds what is the most common bleeding disorder?

A

ITP

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49
Q

what are 3 acquired bleeding disorders?

A

DIC
liver dz
henoch Schonlein Pupura (HSP)

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50
Q

what age range is ITP most common in?

A

2-5

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51
Q

what does ITP most often follow?

A

viral infection

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52
Q

how do you tx ITP

A

steroids

IVIG in severe situations

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53
Q

will there be LAD or HSM with ITP

A

No

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54
Q

what will platelet counts be below with ITP

A

<10,000

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55
Q

What will be increased in the bone marrow with ITP?

A

megakaryoctyes

56
Q

factor VIII deficieicny is what?

A

Hemophilia A

57
Q

factor IX deficieincy is what

A

hemophilia B or

Christmas Dz

58
Q

hemophilias will have prolonged what?

A

aPTT

59
Q

Tx for hemophilia A (classic)?

A

Factor VIII

desmopressin releases endothelial stores of factor VIII and vWf

60
Q

Tx for hemophilia B (christmas dz)?

A

Factor IX

61
Q

Most common inherited bleeding disorder among Caucasians – autosomal dominant

A

Von Willebrand Dz

62
Q

Tx for Von Wilebrand Dz?

A

Desmopressin (causes release of VWF from endothelial stores)

63
Q

what is an autoimmune mediated vasculitis.

A

Henoch schonlein purpura

64
Q

what causes cholestasis?

A

IV fluids (TPN live on trashes your liver)

65
Q

what contains all the clotting factors and can help with DIC?

A

fresh frozen plasma

66
Q

what do you give for a low fibringoen?

A

cryoprecipitate

67
Q

what is a physiologic cause of unconjugated hyperbilirubinemia?

A

breast milk jaundice

68
Q

what enzyme catalyzes bilirubin conjugation in liver.

A

Glucuronyl transferase (low levels at birth)

69
Q

what is a condition where an infant often has polycythemia at birth?

A

trisomy 21

70
Q

caused by Abnormal gene promoter leading to decreased transcription of mRNA less enzyme

A

Gilbert’s syndrome

71
Q

what are sxs of gilbert’s syndrome?

A

life-long episodes of mild juandice

enhanced neonatal juandice

72
Q

tx for gilbert’s syndrome

A

no tx except in neonate w/ excessive hyperbilirubinemia

73
Q

who is Gilbert’s syndrome more common in?

A

males

74
Q

are LFTs normal with Gilberts

A

yes, except direct bili

75
Q

syndrome with no enzyme activity leading to severe unconjugated hyperbilirubinemia starting at th-5th day of life

A

Type 1

Crigler-Najjar Syndrome

76
Q

How do you tx type 1 crigler-najiar syndrome?

A

liver transplant

77
Q

what type of Crigler-Najjar Syndrome has diminished activity?

A

Type 2

78
Q

how do you tx Crigler-Najjar Syndrome

type 2?

A

phenobarbital

79
Q

conjugated hyperbilirubinemia is a sign of what?

A

liver dz

80
Q

what happens with biliary atresia

A

conjugate the bilirubin but you can’t excrete it. Will have impeded bile acid flow

81
Q

what does a wide mediastinum indicate?

A

thymus, lymphadenopathy

82
Q

2 main cancers in peds population

A

leukemias

brain tumors

83
Q

what is the most common death by disease in children?

A

cancer

84
Q

what are the 2 main things to do for a CA workup?

A

CBC with diff

peripheral blood smear

85
Q

what is the most common of the leukemias?

A

acute lymphoblastic leukemia (ALL)

86
Q

when is ALL common?

A

ages 2-10

87
Q

what conditions have a higher incidence of ALL

A

T21
NF type 1
Fanconi’s anemia

88
Q

what will you see with ALL on chest xray?

A

mediastinal widening or anterior mediastinal mass or tracheal compression from lymphadenopathy.

89
Q

are bone marrow transplants (HSCT) common with ALL?

A

no, chemo usually cures

90
Q

which has a worse prognosis- ALL or AML?

A

AML

91
Q

what leukemia can have translocation of chromosome 9 and 22 (Philadelphia chromosome)

A

Chronic Myelogenous Leukemia

92
Q

what is the only curative intervention for CML?

A

HSCT

93
Q

where do lymphomas arise?

A

lymph nodes
thymus
spleen

94
Q

what is the most common lymphoma?

A

Hodgkin disease

95
Q

where will you find a mass with hodgkin lymphoma?

A

cervical, supraclavicular adenopathy or mediastinal

96
Q

what cells will be present with hodgkin?

A

Reed Sternberg cells (malignant B cells)

97
Q

Tx for hodgkin dz?

A

chemo + los dose radiation

98
Q

what will a child with hodgkin present with?

A 
fatigue
cough
SOB
anorexia
weight loss
fever
night sweats
99
Q

are hogkin’s or NH more malignant?

A

NK

100
Q

Tx for NH lymphoma?

A

chemotherapy

101
Q

what is the second most common cancer in kids?(more common solid tumor)

A

brain tumors

102
Q

what is the classic triad of brain tumors in kids?

A

morning headache, vomiting, papilledema

103
Q

what is the most common brain tumor of childhood?

A

astrocytoma (also known as gliomas)

104
Q

symptoms that go along with mass in supratentorial area?

A

seizures
hemiplegia
focal neurological signs

105
Q

What symptoms will you have with an craniopharyngioma (midline tumor)

A

bitemporal hemianopsia
pituitary failure (growth failure)
diabetes insipids
weight gain

106
Q

what would a tumor in the cerebellar and IVth ventricle present with?

A

truncal ataxia
coordination difficulties
abnormal eye movements

107
Q

What would a brainstem tumor present like?

A

cranial nerve defects
pyramidal tract signs
ataxia
often no raised ICP

108
Q

does cerebellar astrocytoma have high or low cure rate?

A

high cure rate

109
Q

what brain tumors have a very poor prognosis?

A

brainstem gliomas

glioblastoma

110
Q

with kidney problem what diagnostic should you start with?

A

Ultrasound

111
Q

what does a neuroblastoma do that a nephroblastoma doesn’t?

A

crosses the midline

112
Q

what is the most common solid neoplasm outside the CNS?

A

Neuroblastoma

113
Q

when you hear abdominal pain and masses what should you suspect?

A

neuroblastoma

114
Q

Tx for neuroblastoma

A

surgical resection with chemo

115
Q

what is the definitive diganostic tool for neuroblastoma?

A

biopsy

116
Q

large tumor in the kidney that doesn’t cross the midline.

A

Wilms tumor

117
Q

what is the 2nd most common abdominal tumors in children?

A

Wilms tumor

118
Q

when are Wilm tumors common (age wise)?

A

2-5, unusual after age 6

119
Q

who can wilms tumors been more common in?

A

Kids with hypospadias, GU malformations and others

120
Q

is a Wilms tumor painless or painful?

A

painless

121
Q

Tx for wilms tumor.

A

surgical resection/ exploration then chemo

122
Q

what other physical symptoms may be present with a Wilms tumor?

A

hematuria and HTN

123
Q

neuroectodermal malignancy” arising from embryonic retinal cells

A

Retinoblastoma

124
Q

most common sign of retinoblastoma.

A

white pupillary reflex

125
Q

tx for retinoblastoma

A

radiation, enucleation (take out eye)

prognosis depends on spread beyond globe

126
Q

when is a retinoblastoma usually diagnosed?

A

before age 3

127
Q

Diagnostics for retinoblastoma

A

MRI

128
Q

most common soft tissue sarcoma in childhood

A

Rhabdomyosarcoma

129
Q

when are the 2 peaks for sarcomas in children?

A

2-5 years and adolescents

130
Q

most common locations for sarcomas in younger patients.

A

head, neck, and GU

131
Q

most common locations for sarcomas in older patients

A

extremities
trunk
male GU

132
Q

Diagnostics for Rhabdomyosarcoma

A

Xrays
Ct
MRI
(look for tumor and mets)

133
Q

Tx for rhabdomyosarcoma

A

surgery
chemo
radiation

134
Q

when are osteosarcomas and ewing sarcomas most often seen

A

growth spurts

135
Q

is osteosarcoma or Ewing sarcoma more commonly seen?

A

osteosarcoma

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