Hema Flashcards

1
Q

stages of hematopoeisis

A
1. mesoblastic/yolk sac 
begins 10-14th day of gestation
2. liver
begins 6-8wks AOG up to 20-24wks AOG
3. bone marrow
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2
Q

pallor apparent at

A

7-8 g/dL

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3
Q
autosomal dominant
macrocytic anemia, low retic
anemia at 2-6mo
snub nose
hypertelorism
flattening of thenar prominence
triphalengeal thumb
abscence of radial pulse
A

diamond blackfan
or
congenital hypoplastic anemia

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4
Q

increase in chromosomal breaks when lymphocytes exposed to alkylating agents

A

fanconi anemia

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5
Q

tx of diamond blackfan

A

steroids

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6
Q
macrocytic anemia
failure to thrive
panceratic fibrosis
insulin dependent diabetes
muscle and neuro impairment
villous atrophy with chronic diarrhea
early death
A

pearson syndrome

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7
Q

most common acquired red cell aplasia in childhood

A

transient erythroblastopenia of childhood

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8
Q

physiologic anemia of infancy at

A

8-12 weeks

at 11g/dL

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9
Q

physiologic anemia in prematures

A

3-6weeks

7-9 g/dL

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10
Q

reticulocytosis in IDA tx occur

A

48-96 hours

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11
Q

expect hg to increase by __ once iron given over __wks

A

1-2g/dL

4weeks

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12
Q

defect in spherocytosis

A

spectrin

ankyrin

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13
Q

Cytoskeletal protein defects
Often involve vertical interactions
of spectrin ankyrin, protein 3

A

Hereditary spherocytosis

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14
Q

lab results Hereditary spherocytosis

A
Spherocytes on blood film
Negative Coombs test
eliminates immune hemolysis
Increased incubated osmotic
fragility
Abnormal cytoskeletal protein
analysis
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15
Q

Primary acquired marrow disorder
RBCs unusually sensitive to
complement-mediated lysis

A

Paroxysmal nocturnal

hemoglobinuria

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16
Q

lab result Paroxysmal nocturnal

hemoglobinuria

A
Decreased WBC CD55 and
CD59 or decreased RBC CD59
by flow cytometry
Marrow aspirate and biopsy to
assess cellularity
Decreased decay-accelerating
factor
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17
Q

warm antibody

A

igG

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18
Q

cold antibody

A

igM

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19
Q
Absence of apolipoprotein β 
Acanthocytes on blood film 
Vitamin E deficiency and
heightened sensitivity to
oxidative damage
A

Abetalipoproteinemia

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20
Q

tx Abetalipoproteinemia

A

vit ADEK
folic acid
dietary restriction triglyerides

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21
Q

hemoglobin S due to

A

single base pair change
thymine for adenine
encodes VALINE instead of GLUTAMINE

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22
Q

sickle cell prophylaxis

A

oral pen V K 125mg bid up to 3yrs then

250mg bid up to 5 years old

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23
Q

osteomyelitis pathogen in sickle cell

A

salmonella

staph

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24
Q

splenic sequestration

A
engorgement of spleen
increase in size
hypovolemia
drop Hg by > 2 g/dL
thrombocytopenia
reticulocytosis
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25
Q

tx to reduce pain episodes in sickle cell

A

hydroxyurea

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26
Q

tx of priapism >4hrs sickle cell

A

aspiration of blood from corpora cavernosa

injection of dilute epinephrine

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27
Q

PRES or
reversible leukoencephalopathy syndrome associated with

headache, confusion, seizures and visual loss

A

sickle cell

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28
Q

management for sickle cell kid with acute focal neuro deficit

A

oxygen to keep sats >96
blood transfusion w/in 1 hr to keep Hg > 10g/dL
exchange transfusion (manual or erythrocytapharesis)
CT scan or MRI

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29
Q

goal in blood transfusion tx in sickle cell

A

keep Hg S < 30% in the first 2 yrs after new stroke then 50% after

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30
Q

tx in ACS sickle cell

A

macrolide

third gen cephalosporin

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31
Q

pathogen ACS in sickle cell

A

strep
mycoplasma pneumoniae
chalmydia

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32
Q

cyanosis visible at methemoglobin level

A

1.5g/24 hr
or
15%

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33
Q

methemoglobin lethal at

A

70%

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34
Q

tx methemoglobinemia

A

methyl blue if toxic

vit C if not toxic looking patient

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35
Q

methyl blue should not be given to

A

g6pd

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36
Q

barts hemoglobin

A

4 gamma globin

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37
Q

hemoglobin h

A

4 beta globin

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38
Q

cooley anemia

b thalassemia major

A

4 alpha globin

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39
Q

features thalassemia

A

maxilla hyperplasia
frontal bossing
flat nasal bridge

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40
Q

endocrine abnormality in thalassemia

A

hypothyroidism
hypogonadotropic gonadism
hypoparathyrodism
diabetes mellitus

41
Q

iron chelators

A

deferoxamine

deferasirox

42
Q

alpha thalassemia trait

A

deletion of 2 alpha globin

43
Q

silent alpha thalassemia

A

deletion of 1 alpha globin

44
Q

episodic hemolytic anemia

A

g6pd deficiency

45
Q

heinz body

A

hemolysis

like g6pd deficiency

46
Q

most common cause of drug-immune hemolytic anemia.

A

Cephalosporin

47
Q

concomitant immune thrombocytopenic purpura with autoimmune hemolytic anemia

A

Evans syndrome

48
Q

Warm antibody

A

IgG

49
Q

Cold antibody

A

IgM

50
Q

When to stop Prednisone in autoimmune hemolytic anemia

A

once the direct Coombs test result becomes negative.

51
Q

Tx in cold autoimmune hemolytic anemia

A

Rituximab

52
Q
hyperpigmentation
cafe au lait
vitilogo
panyctopenia
absent thumbs
horseshoe or absent kidneys
short
hypogonadal
A

fanconi anemia

53
Q

cbc monitoring in fanconi anemia every

A

1-3 months

54
Q

tx for fanconi anemia

A

stem cell

55
Q
pancytopenia
low igG
fialure to thrivve
pancreatic insufficiency - failure of acinar development
short stature
bifid thumb
A

shwachman diamond syndrome

56
Q

refractory sideroblastic anemia
cytoplasmic vacuolization
met acid
exocrine pancreastic insufficiency

A

pearson syndrome

57
Q

reticulate skin pigmentation
mucosal leukoplakia
nail dystrophy
bone marrow failure

A

Zinsser-Cole-Engman syndrome
or
Dyskeratosis congenita

58
Q

dx for paroxysmal nocturnal hemoglobinuria

A

CD55

CD 59

59
Q

loose joints
lax skin
easy bruising

A

ehlers danlos

60
Q

surface activation of clotting

A

intrinsic

61
Q

tisse facter mediation activation of clotting

A

extrinsic

62
Q

mixing studies result

A

normal plasma added to test plasma 1:1
if normalized PT/PTT after then deficiency in factor
if still abnormal PT/PTT then inhibitor is present

63
Q

hallmark of hemophilic bleeding is

A

hemarthrosis

64
Q

hemostatic level factor VIII

A

> 30 - 40%

65
Q

hemostatic level factor IX

A

> 25 - 30%

66
Q

formula to compute for dose factor VIII

A

%desired (rise in factor VIII) x wt x 0.5

67
Q

formula to compute for dose factor IX

A

%desired (rise in factor IX) x wt x 1.4

68
Q

desmopressin DDAVP for

A

hemophilia A factor VIII

69
Q

most common hereditary bleeding disorder

A

von willebrand disease

70
Q

von willebrand disease if VWF levels

A

<30 IU

71
Q

__ can increase VWF and factor VIII level

A

desmopressin DDAVP

72
Q

rapidly spreading purpuric lesions from thromboses of small dermal vessels followed by bleeding into skin

A

purpura fulminans

73
Q

management purpura fulminans

A

FFP

74
Q

venous and arterial thromboses

deficiency in cystathione b synthase

A

homocystinuria

75
Q

tx homocystinuria

A

vit B6

76
Q

factor VIII is also considered as

A

acute phase reactant

77
Q

most common thrombotic event in neonates

A

renal artery thrombosis

78
Q

t or f

heparin crossed the placenta

A

false

79
Q

heparin contraindicated in

A
bacterial endocarditis
cns bleed
bleed in the body
recent eye, brain, spine surgery
lumbar puncture
80
Q

heparin can be neutralized with

A

protamine sulfate

81
Q

warfarin affects

A

factor 10, 9, 7, 2
protein c
protein s

82
Q

warfarin effect enhanced by

A
antibiotics
anabolic steroids
chloral hydrate
laxatives
allopurinol
methylphenidate hydrochloride
vitamin e
83
Q

warfarin effect diminished by

A

oral contraceptive
barbiturates
vit k
phenytoin

84
Q

t or f

warfarin crosses placenta

A

true

85
Q

what clotting factor is NOT produced in the liver

A

factor VIII 8

86
Q

most common acquired anticoagulat inhibitor

A

lupus anticoagulant

87
Q
thrombocytopenia with small platelets
eczema
recurrent infection
x linked
WAS protein
A

wiskott aldrich

88
Q

tx of ITP

A

IVIG 0.8g to 1g/kg/day for 1-2 days

89
Q

drugs that cause thrombocytopenia

A
valproin acid
phenytoin
carbamazepine
sulfonamide
cotrimoxazole
vancomycin
90
Q
fever
microangiopathic hemolytic anemia
thrombocytopenia
abnormal renal fxn
CNS changes
A

thrombotic thrombocytopenic purpura

91
Q

ttp cause

A

acquired deficiency of metalloproteinase (ADAMTS 13)

92
Q

giant hemangioma
localized intravascular coagulation
thrombocytopenia
hypofibrinogenemia

A

kasabach meritt syndrome

93
Q

thrombocytopenia (absent/hypoplasia megarkaryocytes)
absent radius
cows milk allergy (diarrhea, eosinophilia, leukemoid rxn)

A

TAR

thrombocytopenia absent radius syndrome

94
Q

torch infection that causes thrombocytopenia

A

rubella
cmv
syphilis
toxoplasmosis

95
Q

MOA of aspirin

A

irreversibly acetylates cyclooxygenase (impt in thromboxane)

96
Q

absence of VWF/glycoprotein Ib receptor

A

bernard soulier syndrome

97
Q

defect in platelet aggregation (deficiency in platelet fibrinogen receptor)

A

glanzmann thrombastenia

98
Q

tx for platelet dysfxn

A

desmopressin

platelet transfusion