Heme/Onc/Bili Flashcards
(122 cards)
1
Q
what do stem cells originate from?
A
mesoderm
2
Q
what is the early site of hematopoeisis
A
secondary yolk sac
3
Q
when do blood cells appear?
A
16-19 days
4
Q
when does secondary yolk sac regress?
A
GA 10 weeks
5
Q
when does liver begin hematopoiesis?
when does it become the primary site?
A
5-6 wk GA
6-22 wk GA
6
Q
when does BM begin hematopoiesis?
when does it become the primary site?
A
8-19 weeks
> 22 weeks
7
Q
when does erythropoeisis exceed granulopoeisis?
A
10-11 weeks but then granulopoiesis takes over after 12 week
8
Q
what cell types are present in yolk sac?
A
primitive erythrocytes
mature macrophages
9
Q
how does RBC number change with GA
A
increases
10
Q
how does Hct change with GA
A
increases
11
Q
how does MCV change with GA
A
decreases
12
Q
when does retic% peak?
A
peaks at 26-27 weeks then declines
13
Q
how does nucleated RBC change with GA
A
decreases
14
Q
alpha and beta genes of globin are on what chromosomes?
A
16 and 11
15
Q
what hemoglobin changes are present in yolk sac?
A
Hb Gower 1
Hb Gower 2
Hb Portland
16
Q
change in alpha and beta globin after birth
A
alpha stable
beta increases
17
Q
point mutation in sickle cell
A
valine for glutamic acid at position 6
18
Q
MC hemoglobinopathy
A
hemoglobin e
point mutation on beta globin glu –> lys
19
Q
% Hgb F =
and rhogam dose
A
fetal cells/maternal cells x 100
1% = 50 mL
15mL = 1 vials
1 vial = 300 mcg
20
Q
difference of kleir betke and apt test
A
KB =test moms with citric acid-phosphate buffer and adult Hb dissolved out
apt = fetal sample add NaOH; fetal Hgb resists denaturation
21
Q
congenital erythrocyte underproduction
A
- diamond blackfan
- fanconi anemia
22
Q
diamond black fan pathophys
A
AD or AR
pure red cell aplasia
elevated i (vs I) antigen
23
Q
fanconi anemia pathoyphys
A
AR
chromosomal instability
24
Q
fanconi anemia tests
A
mitomycin c - for chromosomal breaks
25
fanconi management
androgens
hematopoeitic growth factors
BMT
26
acquired erythroycyte underproduction
parvob19
aplastic anemia - maternal azathioprine, chloramphenicol
infectious - hepatitis, hiv, syphillis
iron, folic acid, vit b12 deficiency
transient erythroblastopenia of childhood
increase destruction
27
what is the clinical presentation of transient erythroblastopenia of childhood
usually second year
normocytic anemia with low retic count
absent fetal hemoglobin and i antigen
28
basophillic stippling
lead poisoning
iron deficiency
hemolytic
thalassemia
29
blister cells
G6PD
30
elliptocytes
rbc membrane defect
31
heinz bodies
hemolytic
enzymatic
seen in newborns
32
howell jolly bodies
spleen dysfunction or absence
33
hypochromia
iron def
thalassemia
lead poisoning
34
polychromasia
normal in newborns
35
schistocytes
microangiopathic hemolytic anemia
36
when does kidney start making EPO?
initially liver until third trimester when kidney starts making; kidney predominates after birth
37
ABO incompatibility and Rh incompatibility in subsequent pregnancies
Rh but not ABO is worse
38
Rh antigens after D that cause hemolytic disease
c, C, e, E
39
minor blood groups that cause hemolytic disease
Kell (K and k)
Duffy (Fy^a)
Kidd (Jk^a and Jk^b)
40
minor blood groups that DO NOT cause hemolytic disease
Lewis antigen - but causes Coombs results positive
anti I
anti-Fy^b
41
hereditary spherocytosis pathophys
- AD
- UDPGT1; more pronounce jaundice
- defect in membrane proteins (spectrin, ankyrin, band 3 and protein 4.2) leading to instability and splenic sequestration
42
G6PD pathophys
- XLR
- M>F
- enzyme defect in
G6P + NADP -- (G6PD) --> Pentose phosphate + NADPH
43
Pyruvate kinase pathophys
- AR, northern european
- embden-meyerhof pathway and pentose phosphate shunt
- ADP + Phosphoenolpyruvate -- (pyruvate kinase) --> ATP + Pyruvate
44
what is MCC and second MCC RBC enzyme defect
1. G6PD
2. pyruvate kinase
45
blood volume exchange in partial exchange transfusion =
(observed - desired:55-60)/observed x infant blood volume
46
methemoglobinemia pathophys
- iron oxidized or ferric state; does not complex with O2 > decreased O2 carrying capacity
- normally 3%
47
congenital methemoglobinemia
- NADH-MET Hg reductase deficiency (AR, Navajo)
- hemoglobin M (AD, stabilized ferric form; resistant to methylene blue)
48
when are platelets detected in the liver and circulatory system?
8 weeks
49
size of fetal vs adult megakaryocytes
fetal is smaller, but more circulating
50
Neonatal alloimmune thrombocytopenia
- maternal alloantibodies against paternally inherited antigens
HPA-1a MCC
HPA-5b also
51
NAIT vs ITP platelet count of mother
NAIT - mother normal
ITP - low
52
recurrence of NAIT
90%
53
neonatal autoimmune thromboyctopenia
maternal antiplatelet antibodies caused by ITP, lupus other AI disease
--- even if well controlled and after splenectomy --- antibodies still there so infants may develop
therefore maternal platelet count not a good indicator of severity
54
TAR pathophys
AR
absent reduced megakaryocytes
55
CHD a/w TAR
ToF
ASD
56
Amegakaryocyte thrombocytopenia
BM with absent or scarce megakaryocytes
XLR
57
Lab data of decreased platelet production
- normal MPV
- low immature platelet fraction
- low reticulated platelets < 2%
- high TPO > 500
- few megakaryocytes in BM
58
Lab data of increased platelet destruction
- high MPV
- high immature platelet fraction
- high reticulated platelets > 10%
- low/ normal TPO <250
- many megakaryocytes in BM
59
who makes fetus's clotting factors
fetus
maternal clotting factors do not cross placenta
60
when does fetus start making clotting proteins?
5-10 weeks
61
when do clotting levels become comparable to adult levels?
6 months
62
in comparison to adults how much factor XI, XII, prekallekrein and HMW kinonogen do fetuses have
<70%
63
in comparison to adults how much vit K dependent clotting factors do fetuses have
<70%
64
in comparison to adults how much V, VIII, XIII, vWF and fibrinogen do fetuses have?
> 70%
65
what factors are vit K dependent
2, 7, 9, 10
C and S
66
in comparison to adults how much ATIII, protein c and protein s do fetuses have
30%
67
in comparison to adults how much thrombomodulin do fetuses have?
3x
68
prolonged bleeding time ddx
platelets < 100k
platelet function disorder
vW disease
other platelet vessel interaction factors
69
increased MPV
ITP
70
prolonged extrinsic pathway (PT)
inherited factor 7 deficiency
liver dz
DIC
vit K deficiency (2, 7, 9, 10)
inherited defect in factor 5, 10 ,2 if also prolonged PTT
71
prolonged intrinsic pathway (PTT)
contact factors deficiency (XI, XII, PK, HMWK)
hemophillia A (8), B (9)
vWd
factor 5, 10 ,2 inherited/acquired
vit k deficiency
liver
dic
heparin
lupus anticoagulant
72
increased thrombin time
heparin contamination
decreased fibrinogen < 100 mg
abnormal fibrinogen
increased fibrin split products
73
abnormal fibrinogen level
inherited deficiency
>>type 1 - actual
>>type 2 - dysfibrinogenemia
acquired (dic, liver or thrombolytic)
74
bleeding, sick, decreased platelets
prolonged PT and PTT
DIC
75
bleeding, sick, decreased platelets
normal PT and PTT
platelet consumption - infection, nec, renal vein thrombosiss
76
bleeding, sick, normal platelets
prolonged PT and PTT
liver
77
bleeding, sick, normal platelets,
normal PT and PTT
compromised vascular integrity: hypoxia, acidosis
78
bleeding, healthy, decreased platelets
normal PT and PTT
ITP
occult infection
thrombosis
BM hypoplasia/infiltration
79
bleeding, healthy, normal platelets
increased PT and PTT
vit K deficient
80
bleeding, healthy, normal platelets
normal PT and prolonged PTT
hereditary clotting factor deficiencies
81
bleeding, healthy, normal platelets
normal PT and PTT
local bleeding - trauma
platelet abnormalities
factor 8
vWD
82
hemophilia A pathophys
- XR
- factor 8 deficiency
- prolonged PTT
- severe < 1%, moderate 1-5% mild > 5%
- tx: replace factor 8
83
hemophilia B pathophys
XR
factor 9 deficiency
tx: replace factor 9
84
hemophilia C pathophys
- AR, Ashkenazi; Noonan
- factor 11 deficiency
- GU bleeding
- tx: FFP
85
factor 13 deficiency
- AR
- umbilical stump or circumcision site bleeding
- tx: factor 13 or cryo
86
vWD
- AR or AD
- component of factor 8 - ligand between platelet and vessel
- prolonged bleeding time +/- prolonged PTT
- normal PT and platelets
87
how to diagnose vWF
ristocetin factor
88
how to manage vWD
cryo
factor 8 with vWF
DDAVP
89
hemorrhagic disease of newborn pathophys
vit k esssential for 2. 7. 9 .10 and C, S
90
early hemorrhagic
- within 24 hours
- placental transfer of maternal drugs: carbamazepine, phenytoin, barbiturates, cephalosporins, rifampin, isoniazid and warfarin
91
classic hemorrhagic
- 2-7d
- inadequate vit K; esp breast fed
- GI bleeding, umbilical cord, IVH, prolonged bleeding after phlebotomy or circ
92
late hemorrhagic
- 2w-6months
- inadequate intake or hepatobiliary disease
- risk of IVH and death
- M>F
- summer
93
labs in hemorrhagic disease
prolonged PT
normal PTT, platelets -- PTT may prolong if extended time vit K def
94
management of hemorrhagic disease
vit K IM; oral does not prevent
symptoms usually resolve in 4 hours
95
risk of congenital leukemia
- 5/1,000,000
- fanconi, diamond blackfan and tri 21 increase risk
96
histiocytosis pathophys
tissue infiltration by monocytes or macrophages
97
types of diseases with histiocytosis
- letterer-siwe
- langerhan cell histiocytosis
- familial hemophagocytic lymphohistiocytosis (AR)
- viral HLH (CMV, EBV, HSV, adeno)
98
MC solid tumor
1. teratoma
2. neuroblastoma
99
sites of teratomas
sacrococcygeal > head and neck
<10% malignant
100
sites of neuroblastoma
adrenal 70% (neural crest origin)
better prognosis if infantile
101
prognosis factors of neuroblastoma
stage, age, N-myc amplification
stage IV-S, age < 1 and localized to primary tumor with limited mets to liver, skin or BM is favorable
102
pheochromocytoma a/w
- NF
- vonhippel lindau
- islet cell adenoma
- medullary carcinoma of thyroid
103
wilms prognosis
good
104
hepatoblastoma labs
increased AFP
105
retinoblastoma frequency
1/20,000
AD 40%, sporadic 60%
unilaleral 70%, bilateral 30%
106
secondary malignancies of retinoblastoma
- osteosarcoma
- pinealblastoma (inherited)
107
risks of recurrenceof RB
bilateral parent --> 45% child
unilateral parent --> 6% child
bilateral child --> 3% sibling
unilateral child --> 0.4% sibling
108
risk of transfusion infections
HIV 1 in 1.5 million
HBV 1 in 1 million
HCV 1 in 1.2 million
bacterial contamination 1 in 5000 platelet units
109
what to use cryo for?
factor 8 and vWD
110
comparison of peak bili in physiologic jaundice between term and preterm
peak in term: 3-5 days
peak in preterm: 5 days
111
risk of physiologic jaundice
1/200
112
what is configurational isomerization
isomer changes form 4Z, 15Z to 4Z,15E which is less toxic
bilirubin --> bile
113
what is structural isomerizations
the formation of lumirubin
114
phototherapy dose depends on:
1. spectrum: blue 460-490
2. irradiation: 10uW/cm2/cm standard; 30 intensive
3. exposure
4. distance: optimal = 10-15 cm
| SIDE
115
when doing exchange transfusion what should the hct of replacement blood be?
50-55
116
how many aliquots of blood for exchange
5-20
117
how much of the blood volume is replaced?
87%
118
how does ivig help jaundice
bind Fc receptor on reticuloendothelial cells so destruction of RBCs does not occur
119
how does phenobarbital help jaundice
- increase ligandin concentration, increasing uptake
- inducing enzymes
- increasing bile flow
120
how does agar help jaundice
decrease enterohepatic circulation
121
how does metalloporphyrins help jaundice
inhibit heme oxygenase
122
bilirubin pathway
hemoglobin (hemeoxygenase)
heme + goblin
biliverdin (biliverdin reductase)
bilirubin [CO --> carboxyHgb] (glucuronyltransferases)
bilirubin mono and diglucuronides (glucuronidases)
stercobilinogen + urobilinogen
