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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
characteristocs of fanconis anemia (7)
``` kids >3yo pancytopenia (macrocytic anemia) abnl skin pigmentation growth retardation renal abnl absent/hypoplastic thumb risk of AML ```
26
retic count with diamond blackfan
low
27
retic count with transient erythroblastopenia of childhood
low
28
characteristics of diamond blackfan anemia
``` 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 ```
29
treatment of diamond blackfan anemia
transfuse BM
30
charactersiitics of transient erythroblastopenia of childhood
anemia (other cell lines OK) see at 18-26 mos resolves on its own
31
what is the ANC in mild neutropenia
1000-1500
32
what is the ANC in moderate neutropenia
500-1000
33
what is the ANC in severe neutropenia
<500
34
recurrent mucosal ulcerations
neutropenia
35
hematological side effect of macrolide
neutropenia
36
what is the inheritance of cyclic neutropenia
AD
37
what is the treatment for cyclic neutropenia
daily rhG-CSF to reduce the number of neutropenic days
38
what is the inheritance of kostmann agranulocytosis
AR
39
characteristics of kostmann agranulocytosis
severe neutropenia d/t arrest in development of neutrophils
40
what is the treatment for kostmann agranulocytosis
rhG-CSF | stem cell transplant
41
what are the characteristics of Shwachman-diamond syndrome (7)
``` pancytopenia and pancreatic exocrine insufficiency short stature diarrhea/steatorrhea URI and skin infections skeletal abnl (clinodactyly, syndactyly) risk of leukemic transformation ```
42
pancytopenia and pancreatic exocrine insufficiency
Shwachman-diamond syndrome
43
what medications cause thrombocytopenia
AEDs sulfa meds vanco
44
newborn baby with thrombocytopenia and asymptomatic mother with nl labs
neonatal alloimmune thrombocytopenia isolated and transient
45
newborn baby with thrombocytopenia and mother with thrombocytopenia
neonatal autoimmune thrombocytopenia both mom and baby with low plt
46
what is kasabach merritt syndrome
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
what is the inhertiance of hemophilia
XR
48
what is the function of vWF
needed for factor VIII fxn and normal plt aggregation
49
coags with vWF
``` nl PT prolonged PTT (d/t abnl factor 8 fxn) ```
50
tx of von willebrands dz
DDAVP (desmopressin)- increases plasma vWF and factor VIII factor VIII concentrate aminocaprioic a for mucosal bleeding- inhibits fibrinolysis
51
labs in DIC
elevated ddimer | low fibrinogen
52
where does osteogenic sarcoma metastasize to
lungs
53
what is the most common primary malignant bone tumor in children and adolescents
osteogenic sarcoma (osteosarcoma)
54
what bone tumor improves with NSAIDs
osteoid osteoma not actually cancer pain is worse at night can see radiolucency with surround sclerosis
55
where does leukemia tend to relapse
CNS and testes
56
name the malignancy unexplained pruitus
lymphoma
57
CBC in hodgkins lymphoma
elevated WBC with relatively low lymphocytes | + reed sternberg cells
58
name the malignancy persistent scalp or diaper seborrheic rash chronic ear dc or mastoiditis lytic lesions in the skull or vertebral collapse excessive urination
langerhans excessive urination from DI/pituitary involvement
59
how do you dx langerhans
skin biopsy and electron microscopy
60
what is the most common solid tumor of infancy
neuroblastoma
61
what is the most common abdominal mass in infancy
hydronephrosis
62
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 ```
neuroblastoma
63
what is the cause of HTN in neuroblastomas
renal a compression
64
how do you dx neuroblastoma
biopsy urine VMA and HMA (NOT urine catecholamines- epi/norepi) neuroblasts in BM MIBG scan
65
what is the inheritance of retinoblastoma
AD with incomplete penetrance | RB1 on long arm of chromosome 13
66
children with retinoblastoma are at risk for ____ in the future
sarcomas | malignant melanomas
67
what is the most common soft tissue sarcoma
rhabdomyosarcoma
68
what is the most common pediatric abdominal malignancy
wilms tumor
69
what is WAGR
wilms aniridia GU abnl retardation
70
what conditions are a/w wilms
WAGR | beckwidth weideman
71
tumors a/w beckwidth weideman
wilms | hepatoblastoma
72
name the malignancy child with abdominal mass x ray shows calcifications
neuroblastoma wilms tumor does not have calcifications
73
treatment of wilms
remove it | then chemo and radiation
74
how do you prevent tumor lysis syndrome (3)
hydration alkalinization allopurinol
75
why would you now want to intubate a pt with a mediastinal mass
mass is below the vocal cords so if you intubate and paralyze the pt will not be able to breathe
76
what is a normal range for the absolute reticulocyte count
27,000-120,000 retic % x RBC
77
what is the treatment for iron deficiency anemia
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
bernard soulier syndrome
AR deficiency of glycoprotein 1b mild thrombocytopenia with large plt
79
glanzmann thrombasthenia
AR abnormality in alpha2b-beta3 receptor poor plt aggregation
80
what is the most common bleeding do
von willebrands dz
81
name the malignancy well child with HTN and abdominal mass
wilms tumor
82
name the malignancy former premie abdominal mass
hepatoblastoma
83
name the malignancy most common bone tumor
osteosarcoma
84
name the malignancy most common bone tumor with soft tissue involvement
ewings
85
name the malignancy most common soft tissue tumor
rhabdomyosarcoma
86
what is the tx for heart failure 2/2 doxorubicin
ACE inhibitor

heme onc (2 decks)

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