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hemeonc Flashcards

1
Q

when is the physiologic nadir for anemia of the newborn

A

2-3 mos FT

1-2 mos premie

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2
Q

what is the cause of the physiologic nadir in anemia of the newborn

A

decreased epo production

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3
Q

how do you guesstimate nl MCV in children

A

70+2(age)

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4
Q

what are the components of hemoglobin A

A

2 alpha, 2 beta

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5
Q

what are the components of hemoglobin A2

A

2 alpha, 2 delta

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6
Q

what are the components of hemoglobin F

A

2 alpha, 2 gamma

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7
Q

a hgb electrophoresis shows hgb bart, what is this indicative of?

A

alpha thalassemia

when you lack alpha chains, they cannot bind to gamma to make HgbF, therefore you have a bunch of free gamma chains that get together and make hgb bart

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8
Q

what is HgbH

A

when you have 3 defective alpha genes
leads to hemolysis and hepatomegaly
tx with transfusion and splenectomy

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9
Q

what is the result of having 4 defective alpha genes

A

hydrops, still birth, death soon after birth

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10
Q

beta thalassemia minor

A

only 1 beta globulin gene

asymptomatic

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11
Q

what is cooleys anemia

A 
beta thalassemia major- 2 defective beta globulins
have HgbA2 (2 alpha, 2 delta) and HgbF (2 alpha, 2 gamma)
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12
Q

why is it important to give iron supplementation

A

iron deficiency results in delays in cognitive development

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13
Q

what lab findings are c/w lead poisoning

A

microcytic anemia, ringed sideroblast

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14
Q

causes of B12 deficiency (4)

A

1- IF deficiency (pernicious anemia)
2- bacterial overgrowth
3- bowel resection
4- vegetarian diet

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15
Q

what time of anemia do you see in pts who drink goat milk

A

macrocytic
folate deficiency

goats are fools

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16
Q

what is the dx?

anemia with positive coombs

A

immune mediated

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17
Q

heinz bodies

A

G6PD deficiency

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18
Q

what is the inheritance of G6PD deficiency

A

x linked

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19
Q

what is the inheritance of hereditary spherocytosis

A

AD

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20
Q

when should you test a child for G6PD deficiency

A

NOT during or immediately after an acute episode as reticulocytes can have G6PD

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21
Q

what is the most common inherited cause of hemolytic anemia

A

hereditary spherocytosis

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22
Q

treatment of hereditary spherocytosis

A

transfusion

splenectomy is curative

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23
Q

howell jolly body

A

SCD

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24
Q

what is the hematologic side effect of chloramphenicol

A

aplastic crisis

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25
Q

characteristocs of fanconis anemia (7)

A 
kids >3yo
pancytopenia (macrocytic anemia)
abnl skin pigmentation
growth retardation
renal abnl
absent/hypoplastic thumb
risk of AML
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26
Q

retic count with diamond blackfan

A

low

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27
Q

retic count with transient erythroblastopenia of childhood

A

low

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28
Q

characteristics of diamond blackfan anemia

A 
macrocytic anemia (other cell lines OK) with low retic
arrest of maturation of red cells
thumb abnl (triphalangeal thumb)
urogenital defect
craniofacial problem
2-3 mos old
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29
Q

treatment of diamond blackfan anemia

A

transfuse BM

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30
Q

charactersiitics of transient erythroblastopenia of childhood

A

anemia (other cell lines OK)
see at 18-26 mos
resolves on its own

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31
Q

what is the ANC in mild neutropenia

A

1000-1500

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32
Q

what is the ANC in moderate neutropenia

A

500-1000

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33
Q

what is the ANC in severe neutropenia

A

<500

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34
Q

recurrent mucosal ulcerations

A

neutropenia

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35
Q

hematological side effect of macrolide

A

neutropenia

36
Q

what is the inheritance of cyclic neutropenia

A

AD

37
Q

what is the treatment for cyclic neutropenia

A

daily rhG-CSF to reduce the number of neutropenic days

38
Q

what is the inheritance of kostmann agranulocytosis

A

AR

39
Q

characteristics of kostmann agranulocytosis

A

severe neutropenia d/t arrest in development of neutrophils

40
Q

what is the treatment for kostmann agranulocytosis

A

rhG-CSF

stem cell transplant

41
Q

what are the characteristics of Shwachman-diamond syndrome (7)

A 
pancytopenia and pancreatic exocrine insufficiency
short stature
diarrhea/steatorrhea
URI and skin infections 
skeletal abnl (clinodactyly, syndactyly)
risk of leukemic transformation
42
Q

pancytopenia and pancreatic exocrine insufficiency

A

Shwachman-diamond syndrome

43
Q

what medications cause thrombocytopenia

A

AEDs
sulfa meds
vanco

44
Q

newborn baby with thrombocytopenia and asymptomatic mother with nl labs

A

neonatal alloimmune thrombocytopenia

isolated and transient

45
Q

newborn baby with thrombocytopenia and mother with thrombocytopenia

A

neonatal autoimmune thrombocytopenia

both mom and baby with low plt

46
Q

what is kasabach merritt syndrome

A

hemangioma that is a sand trap for plt
thrombocytopenia and consumptive coagulopathy
risk of DIC

think of a merrit on your back causing you to have a black and blue hemangioma!

47
Q

what is the inhertiance of hemophilia

A

XR

48
Q

what is the function of vWF

A

needed for factor VIII fxn and normal plt aggregation

49
Q

coags with vWF

A 
nl PT 
prolonged PTT (d/t abnl factor 8 fxn)
50
Q

tx of von willebrands dz

A

DDAVP (desmopressin)- increases plasma vWF and factor VIII
factor VIII concentrate
aminocaprioic a for mucosal bleeding- inhibits fibrinolysis

51
Q

labs in DIC

A

elevated ddimer

low fibrinogen

52
Q

where does osteogenic sarcoma metastasize to

A

lungs

53
Q

what is the most common primary malignant bone tumor in children and adolescents

A

osteogenic sarcoma (osteosarcoma)

54
Q

what bone tumor improves with NSAIDs

A

osteoid osteoma

not actually cancer
pain is worse at night
can see radiolucency with surround sclerosis

55
Q

where does leukemia tend to relapse

A

CNS and testes

56
Q

name the malignancy

unexplained pruitus

A

lymphoma

57
Q

CBC in hodgkins lymphoma

A

elevated WBC with relatively low lymphocytes

+ reed sternberg cells

58
Q

name the malignancy

persistent scalp or diaper seborrheic rash
chronic ear dc or mastoiditis
lytic lesions in the skull or vertebral collapse
excessive urination

A

langerhans

excessive urination from DI/pituitary involvement

59
Q

how do you dx langerhans

A

skin biopsy and electron microscopy

60
Q

what is the most common solid tumor of infancy

A

neuroblastoma

61
Q

what is the most common abdominal mass in infancy

A

hydronephrosis

62
Q

name the malignancy

persistent bone/joint pain
non tender abdominal mass
wt loss, anorexia, night sweats, fever
UTI
racoon eyes and proptosis
horner syndrome
irritability, HTN, diarrhea
opsoclonus myoclonus
A

neuroblastoma

63
Q

what is the cause of HTN in neuroblastomas

A

renal a compression

64
Q

how do you dx neuroblastoma

A

biopsy
urine VMA and HMA (NOT urine catecholamines- epi/norepi)
neuroblasts in BM
MIBG scan

65
Q

what is the inheritance of retinoblastoma

A

AD with incomplete penetrance

RB1 on long arm of chromosome 13

66
Q

children with retinoblastoma are at risk for ____ in the future

A

sarcomas

malignant melanomas

67
Q

what is the most common soft tissue sarcoma

A

rhabdomyosarcoma

68
Q

what is the most common pediatric abdominal malignancy

A

wilms tumor

69
Q

what is WAGR

A

wilms
aniridia
GU abnl
retardation

70
Q

what conditions are a/w wilms

A

WAGR

beckwidth weideman

71
Q

tumors a/w beckwidth weideman

A

wilms

hepatoblastoma

72
Q

name the malignancy

child with abdominal mass
x ray shows calcifications

A

neuroblastoma

wilms tumor does not have calcifications

73
Q

treatment of wilms

A

remove it

then chemo and radiation

74
Q

how do you prevent tumor lysis syndrome (3)

A

hydration
alkalinization
allopurinol

75
Q

why would you now want to intubate a pt with a mediastinal mass

A

mass is below the vocal cords so if you intubate and paralyze the pt will not be able to breathe

76
Q

what is a normal range for the absolute reticulocyte count

A

27,000-120,000

retic % x RBC

77
Q

what is the treatment for iron deficiency anemia

A

oral elemental iron 3-6 mg/kg/d x 4weeks
then retest
if increase of hgb 1 or hct by 3%
continue therapy for 2 mos and recheck

78
Q

bernard soulier syndrome

A

AR
deficiency of glycoprotein 1b
mild thrombocytopenia with large plt

79
Q

glanzmann thrombasthenia

A

AR
abnormality in alpha2b-beta3 receptor
poor plt aggregation

80
Q

what is the most common bleeding do

A

von willebrands dz

81
Q

name the malignancy

well child with HTN and abdominal mass

A

wilms tumor

82
Q

name the malignancy

former premie abdominal mass

A

hepatoblastoma

83
Q

name the malignancy

most common bone tumor

A

osteosarcoma

84
Q

name the malignancy

most common bone tumor with soft tissue involvement

A

ewings

85
Q

name the malignancy

most common soft tissue tumor

A

rhabdomyosarcoma

86
Q

what is the tx for heart failure 2/2 doxorubicin

A

ACE inhibitor

heme onc (2 decks)

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