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
2
Q
pallor apparent at
A
7-8 g/dL
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
4
Q
increase in chromosomal breaks when lymphocytes exposed to alkylating agents
A
fanconi anemia
5
Q
tx of diamond blackfan
A
steroids
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
7
Q
most common acquired red cell aplasia in childhood
A
transient erythroblastopenia of childhood
8
Q
physiologic anemia of infancy at
A
8-12 weeks
at 11g/dL
9
Q
physiologic anemia in prematures
A
3-6weeks
7-9 g/dL
10
Q
reticulocytosis in IDA tx occur
A
48-96 hours
11
Q
expect hg to increase by __ once iron given over __wks
A
1-2g/dL
4weeks
12
Q
defect in spherocytosis
A
spectrin
ankyrin
13
Q
Cytoskeletal protein defects
Often involve vertical interactions
of spectrin ankyrin, protein 3
A
Hereditary spherocytosis
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
15
Q
Primary acquired marrow disorder
RBCs unusually sensitive to
complement-mediated lysis
A
Paroxysmal nocturnal
hemoglobinuria
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
17
Q
warm antibody
A
igG
18
Q
cold antibody
A
igM
19
Q
Absence of apolipoprotein β Acanthocytes on blood film Vitamin E deficiency and heightened sensitivity to oxidative damage
A
Abetalipoproteinemia
20
Q
tx Abetalipoproteinemia
A
vit ADEK
folic acid
dietary restriction triglyerides
21
Q
hemoglobin S due to
A
single base pair change
thymine for adenine
encodes VALINE instead of GLUTAMINE
22
Q
sickle cell prophylaxis
A
oral pen V K 125mg bid up to 3yrs then
250mg bid up to 5 years old
23
Q
osteomyelitis pathogen in sickle cell
A
salmonella
staph
24
Q
splenic sequestration
A
engorgement of spleen increase in size hypovolemia drop Hg by > 2 g/dL thrombocytopenia reticulocytosis
25
tx to reduce pain episodes in sickle cell
hydroxyurea
26
tx of priapism >4hrs sickle cell
aspiration of blood from corpora cavernosa
| injection of dilute epinephrine
27
PRES or
reversible leukoencephalopathy syndrome associated with
headache, confusion, seizures and visual loss
sickle cell
28
management for sickle cell kid with acute focal neuro deficit
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
29
goal in blood transfusion tx in sickle cell
keep Hg S < 30% in the first 2 yrs after new stroke then 50% after
30
tx in ACS sickle cell
macrolide
| third gen cephalosporin
31
pathogen ACS in sickle cell
strep
mycoplasma pneumoniae
chalmydia
32
cyanosis visible at methemoglobin level
1.5g/24 hr
or
15%
33
methemoglobin lethal at
70%
34
tx methemoglobinemia
methyl blue if toxic
| vit C if not toxic looking patient
35
methyl blue should not be given to
g6pd
36
barts hemoglobin
4 gamma globin
37
hemoglobin h
4 beta globin
38
cooley anemia
| b thalassemia major
4 alpha globin
39
features thalassemia
maxilla hyperplasia
frontal bossing
flat nasal bridge
40
endocrine abnormality in thalassemia
hypothyroidism
hypogonadotropic gonadism
hypoparathyrodism
diabetes mellitus
41
iron chelators
deferoxamine
| deferasirox
42
alpha thalassemia trait
deletion of 2 alpha globin
43
silent alpha thalassemia
deletion of 1 alpha globin
44
episodic hemolytic anemia
g6pd deficiency
45
heinz body
hemolysis
| like g6pd deficiency
46
most common cause of drug-immune hemolytic anemia.
Cephalosporin
47
concomitant immune thrombocytopenic purpura with autoimmune hemolytic anemia
Evans syndrome
48
Warm antibody
IgG
49
Cold antibody
IgM
50
When to stop Prednisone in autoimmune hemolytic anemia
once the direct Coombs test result becomes negative.
51
Tx in cold autoimmune hemolytic anemia
Rituximab
52
```
hyperpigmentation
cafe au lait
vitilogo
panyctopenia
absent thumbs
horseshoe or absent kidneys
short
hypogonadal
```
fanconi anemia
53
cbc monitoring in fanconi anemia every
1-3 months
54
tx for fanconi anemia
stem cell
55
```
pancytopenia
low igG
fialure to thrivve
pancreatic insufficiency - failure of acinar development
short stature
bifid thumb
```
shwachman diamond syndrome
56
refractory sideroblastic anemia
cytoplasmic vacuolization
met acid
exocrine pancreastic insufficiency
pearson syndrome
57
reticulate skin pigmentation
mucosal leukoplakia
nail dystrophy
bone marrow failure
Zinsser-Cole-Engman syndrome
or
Dyskeratosis congenita
58
dx for paroxysmal nocturnal hemoglobinuria
CD55
| CD 59
59
loose joints
lax skin
easy bruising
ehlers danlos
60
surface activation of clotting
intrinsic
61
tisse facter mediation activation of clotting
extrinsic
62
mixing studies result
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
hallmark of hemophilic bleeding is
hemarthrosis
64
hemostatic level factor VIII
> 30 - 40%
65
hemostatic level factor IX
>25 - 30%
66
formula to compute for dose factor VIII
%desired (rise in factor VIII) x wt x 0.5
67
formula to compute for dose factor IX
%desired (rise in factor IX) x wt x 1.4
68
desmopressin DDAVP for
hemophilia A factor VIII
69
most common hereditary bleeding disorder
von willebrand disease
70
von willebrand disease if VWF levels
<30 IU
71
__ can increase VWF and factor VIII level
desmopressin DDAVP
72
rapidly spreading purpuric lesions from thromboses of small dermal vessels followed by bleeding into skin
purpura fulminans
73
management purpura fulminans
FFP
74
venous and arterial thromboses
| deficiency in cystathione b synthase
homocystinuria
75
tx homocystinuria
vit B6
76
factor VIII is also considered as
acute phase reactant
77
most common thrombotic event in neonates
renal artery thrombosis
78
t or f
| heparin crossed the placenta
false
79
heparin contraindicated in
```
bacterial endocarditis
cns bleed
bleed in the body
recent eye, brain, spine surgery
lumbar puncture
```
80
heparin can be neutralized with
protamine sulfate
81
warfarin affects
factor 10, 9, 7, 2
protein c
protein s
82
warfarin effect enhanced by
```
antibiotics
anabolic steroids
chloral hydrate
laxatives
allopurinol
methylphenidate hydrochloride
vitamin e
```
83
warfarin effect diminished by
oral contraceptive
barbiturates
vit k
phenytoin
84
t or f
| warfarin crosses placenta
true
85
what clotting factor is NOT produced in the liver
factor VIII 8
86
most common acquired anticoagulat inhibitor
lupus anticoagulant
87
```
thrombocytopenia with small platelets
eczema
recurrent infection
x linked
WAS protein
```
wiskott aldrich
88
tx of ITP
IVIG 0.8g to 1g/kg/day for 1-2 days
89
drugs that cause thrombocytopenia
```
valproin acid
phenytoin
carbamazepine
sulfonamide
cotrimoxazole
vancomycin
```
90
```
fever
microangiopathic hemolytic anemia
thrombocytopenia
abnormal renal fxn
CNS changes
```
thrombotic thrombocytopenic purpura
91
ttp cause
acquired deficiency of metalloproteinase (ADAMTS 13)
92
giant hemangioma
localized intravascular coagulation
thrombocytopenia
hypofibrinogenemia
kasabach meritt syndrome
93
thrombocytopenia (absent/hypoplasia megarkaryocytes)
absent radius
cows milk allergy (diarrhea, eosinophilia, leukemoid rxn)
TAR
| thrombocytopenia absent radius syndrome
94
torch infection that causes thrombocytopenia
rubella
cmv
syphilis
toxoplasmosis
95
MOA of aspirin
irreversibly acetylates cyclooxygenase (impt in thromboxane)
96
absence of VWF/glycoprotein Ib receptor
bernard soulier syndrome
97
defect in platelet aggregation (deficiency in platelet fibrinogen receptor)
glanzmann thrombastenia
98
tx for platelet dysfxn
desmopressin
| platelet transfusion
