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Heme/Onc/Bili Flashcards

1
Q

what do stem cells originate from?

A

mesoderm

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2
Q

what is the early site of hematopoeisis

A

secondary yolk sac

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3
Q

when do blood cells appear?

A

16-19 days

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4
Q

when does secondary yolk sac regress?

A

GA 10 weeks

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5
Q

when does liver begin hematopoiesis?
when does it become the primary site?

A

5-6 wk GA

6-22 wk GA

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6
Q

when does BM begin hematopoiesis?
when does it become the primary site?

A

8-19 weeks

> 22 weeks

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7
Q

when does erythropoeisis exceed granulopoeisis?

A

10-11 weeks but then granulopoiesis takes over after 12 week

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8
Q

what cell types are present in yolk sac?

A

primitive erythrocytes
mature macrophages

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9
Q

how does RBC number change with GA

A

increases

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10
Q

how does Hct change with GA

A

increases

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11
Q

how does MCV change with GA

A

decreases

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12
Q

when does retic% peak?

A

peaks at 26-27 weeks then declines

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13
Q

how does nucleated RBC change with GA

A

decreases

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14
Q

alpha and beta genes of globin are on what chromosomes?

A

16 and 11

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15
Q

what hemoglobin changes are present in yolk sac?

A

Hb Gower 1
Hb Gower 2
Hb Portland

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16
Q

change in alpha and beta globin after birth

A

alpha stable
beta increases

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17
Q

point mutation in sickle cell

A

valine for glutamic acid at position 6

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18
Q

MC hemoglobinopathy

A

hemoglobin e
point mutation on beta globin glu –> lys

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19
Q

% Hgb F =

and rhogam dose

A

fetal cells/maternal cells x 100

1% = 50 mL
15mL = 1 vials
1 vial = 300 mcg

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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

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21
Q

congenital erythrocyte underproduction

A
  1. diamond blackfan
  2. fanconi anemia
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22
Q

diamond black fan pathophys

A

AD or AR
pure red cell aplasia
elevated i (vs I) antigen

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23
Q

fanconi anemia pathoyphys

A

AR
chromosomal instability

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24
Q

fanconi anemia tests

A

mitomycin c - for chromosomal breaks

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25
Q

fanconi management

A

androgens
hematopoeitic growth factors
BMT

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26
Q

acquired erythroycyte underproduction

A

parvob19
aplastic anemia - maternal azathioprine, chloramphenicol
infectious - hepatitis, hiv, syphillis
iron, folic acid, vit b12 deficiency
transient erythroblastopenia of childhood
increase destruction

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27
Q

what is the clinical presentation of transient erythroblastopenia of childhood

A

usually second year
normocytic anemia with low retic count
absent fetal hemoglobin and i antigen

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28
Q

basophillic stippling

A

lead poisoning
iron deficiency
hemolytic
thalassemia

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29
Q

blister cells

A

G6PD

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30
Q

elliptocytes

A

rbc membrane defect

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31
Q

heinz bodies

A

hemolytic
enzymatic
seen in newborns

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32
Q

howell jolly bodies

A

spleen dysfunction or absence

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33
Q

hypochromia

A

iron def
thalassemia
lead poisoning

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34
Q

polychromasia

A

normal in newborns

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35
Q

schistocytes

A

microangiopathic hemolytic anemia

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36
Q

when does kidney start making EPO?

A

initially liver until third trimester when kidney starts making; kidney predominates after birth

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37
Q

ABO incompatibility and Rh incompatibility in subsequent pregnancies

A

Rh but not ABO is worse

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38
Q

Rh antigens after D that cause hemolytic disease

A

c, C, e, E

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39
Q

minor blood groups that cause hemolytic disease

A

Kell (K and k)
Duffy (Fy^a)
Kidd (Jk^a and Jk^b)

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40
Q

minor blood groups that DO NOT cause hemolytic disease

A

Lewis antigen - but causes Coombs results positive
anti I
anti-Fy^b

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41
Q

hereditary spherocytosis pathophys

A
  • AD
  • UDPGT1; more pronounce jaundice
  • defect in membrane proteins (spectrin, ankyrin, band 3 and protein 4.2) leading to instability and splenic sequestration
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42
Q

G6PD pathophys

A
  • XLR
  • M>F
  • enzyme defect in
    G6P + NADP – (G6PD) –> Pentose phosphate + NADPH
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43
Q

Pyruvate kinase pathophys

A
  • AR, northern european
  • embden-meyerhof pathway and pentose phosphate shunt
  • ADP + Phosphoenolpyruvate – (pyruvate kinase) –> ATP + Pyruvate
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44
Q

what is MCC and second MCC RBC enzyme defect

A
  1. G6PD
  2. pyruvate kinase
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45
Q

blood volume exchange in partial exchange transfusion =

A

(observed - desired:55-60)/observed x infant blood volume

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46
Q

methemoglobinemia pathophys

A
  • iron oxidized or ferric state; does not complex with O2 > decreased O2 carrying capacity
  • normally 3%
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47
Q

congenital methemoglobinemia

A
  • NADH-MET Hg reductase deficiency (AR, Navajo)
  • hemoglobin M (AD, stabilized ferric form; resistant to methylene blue)
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48
Q

when are platelets detected in the liver and circulatory system?

A

8 weeks

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49
Q

size of fetal vs adult megakaryocytes

A

fetal is smaller, but more circulating

50
Q

Neonatal alloimmune thrombocytopenia

A
  • maternal alloantibodies against paternally inherited antigens
    HPA-1a MCC
    HPA-5b also
51
Q

NAIT vs ITP platelet count of mother

A

NAIT - mother normal
ITP - low

52
Q

recurrence of NAIT

A

90%

53
Q

neonatal autoimmune thromboyctopenia

A

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
Q

TAR pathophys

A

AR
absent reduced megakaryocytes

55
Q

CHD a/w TAR

A

ToF
ASD

56
Q

Amegakaryocyte thrombocytopenia

A

BM with absent or scarce megakaryocytes
XLR

57
Q

Lab data of decreased platelet production

A
  • normal MPV
  • low immature platelet fraction
  • low reticulated platelets < 2%
  • high TPO > 500
  • few megakaryocytes in BM
58
Q

Lab data of increased platelet destruction

A
  • high MPV
  • high immature platelet fraction
  • high reticulated platelets > 10%
  • low/ normal TPO <250
  • many megakaryocytes in BM
59
Q

who makes fetus’s clotting factors

A

fetus
maternal clotting factors do not cross placenta

60
Q

when does fetus start making clotting proteins?

A

5-10 weeks

61
Q

when do clotting levels become comparable to adult levels?

A

6 months

62
Q

in comparison to adults how much factor XI, XII, prekallekrein and HMW kinonogen do fetuses have

A

<70%

63
Q

in comparison to adults how much vit K dependent clotting factors do fetuses have

A

<70%

64
Q

in comparison to adults how much V, VIII, XIII, vWF and fibrinogen do fetuses have?

A

> 70%

65
Q

what factors are vit K dependent

A

2, 7, 9, 10
C and S

66
Q

in comparison to adults how much ATIII, protein c and protein s do fetuses have

A

30%

67
Q

in comparison to adults how much thrombomodulin do fetuses have?

A

3x

68
Q

prolonged bleeding time ddx

A

platelets < 100k
platelet function disorder
vW disease
other platelet vessel interaction factors

69
Q

increased MPV

A

ITP

70
Q

prolonged extrinsic pathway (PT)

A

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
Q

prolonged intrinsic pathway (PTT)

A

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
Q

increased thrombin time

A

heparin contamination
decreased fibrinogen < 100 mg
abnormal fibrinogen
increased fibrin split products

73
Q

abnormal fibrinogen level

A

inherited deficiency
»type 1 - actual
»type 2 - dysfibrinogenemia

acquired (dic, liver or thrombolytic)

74
Q

bleeding, sick, decreased platelets
prolonged PT and PTT

A

DIC

75
Q

bleeding, sick, decreased platelets
normal PT and PTT

A

platelet consumption - infection, nec, renal vein thrombosiss

76
Q

bleeding, sick, normal platelets
prolonged PT and PTT

A

liver

77
Q

bleeding, sick, normal platelets,
normal PT and PTT

A

compromised vascular integrity: hypoxia, acidosis

78
Q

bleeding, healthy, decreased platelets
normal PT and PTT

A

ITP
occult infection
thrombosis
BM hypoplasia/infiltration

79
Q

bleeding, healthy, normal platelets
increased PT and PTT

A

vit K deficient

80
Q

bleeding, healthy, normal platelets
normal PT and prolonged PTT

A

hereditary clotting factor deficiencies

81
Q

bleeding, healthy, normal platelets
normal PT and PTT

A

local bleeding - trauma
platelet abnormalities
factor 8
vWD

82
Q

hemophilia A pathophys

A
  • XR
  • factor 8 deficiency
  • prolonged PTT
  • severe < 1%, moderate 1-5% mild > 5%
  • tx: replace factor 8
83
Q

hemophilia B pathophys

A

XR
factor 9 deficiency
tx: replace factor 9

84
Q

hemophilia C pathophys

A
  • AR, Ashkenazi; Noonan
  • factor 11 deficiency
  • GU bleeding
  • tx: FFP
85
Q

factor 13 deficiency

A
  • AR
  • umbilical stump or circumcision site bleeding
  • tx: factor 13 or cryo
86
Q

vWD

A
  • AR or AD
  • component of factor 8 - ligand between platelet and vessel
  • prolonged bleeding time +/- prolonged PTT
  • normal PT and platelets
87
Q

how to diagnose vWF

A

ristocetin factor

88
Q

how to manage vWD

A

cryo
factor 8 with vWF
DDAVP

89
Q

hemorrhagic disease of newborn pathophys

A

vit k esssential for 2. 7. 9 .10 and C, S

90
Q

early hemorrhagic

A
  • within 24 hours
  • placental transfer of maternal drugs: carbamazepine, phenytoin, barbiturates, cephalosporins, rifampin, isoniazid and warfarin
91
Q

classic hemorrhagic

A
  • 2-7d
  • inadequate vit K; esp breast fed
  • GI bleeding, umbilical cord, IVH, prolonged bleeding after phlebotomy or circ
92
Q

late hemorrhagic

A
  • 2w-6months
  • inadequate intake or hepatobiliary disease
  • risk of IVH and death
  • M>F
  • summer
93
Q

labs in hemorrhagic disease

A

prolonged PT
normal PTT, platelets – PTT may prolong if extended time vit K def

94
Q

management of hemorrhagic disease

A

vit K IM; oral does not prevent
symptoms usually resolve in 4 hours

95
Q

risk of congenital leukemia

A
  • 5/1,000,000
  • fanconi, diamond blackfan and tri 21 increase risk
96
Q

histiocytosis pathophys

A

tissue infiltration by monocytes or macrophages

97
Q

types of diseases with histiocytosis

A
  • letterer-siwe
  • langerhan cell histiocytosis
  • familial hemophagocytic lymphohistiocytosis (AR)
  • viral HLH (CMV, EBV, HSV, adeno)
98
Q

MC solid tumor

A
  1. teratoma
  2. neuroblastoma
99
Q

sites of teratomas

A

sacrococcygeal > head and neck
<10% malignant

100
Q

sites of neuroblastoma

A

adrenal 70% (neural crest origin)
better prognosis if infantile

101
Q

prognosis factors of neuroblastoma

A

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
Q

pheochromocytoma a/w

A
  • NF
  • vonhippel lindau
  • islet cell adenoma
  • medullary carcinoma of thyroid
103
Q

wilms prognosis

A

good

104
Q

hepatoblastoma labs

A

increased AFP

105
Q

retinoblastoma frequency

A

1/20,000
AD 40%, sporadic 60%
unilaleral 70%, bilateral 30%

106
Q

secondary malignancies of retinoblastoma

A
  • osteosarcoma
  • pinealblastoma (inherited)
107
Q

risks of recurrenceof RB

A

bilateral parent –> 45% child
unilateral parent –> 6% child
bilateral child –> 3% sibling
unilateral child –> 0.4% sibling

108
Q

risk of transfusion infections

A

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
Q

what to use cryo for?

A

factor 8 and vWD

110
Q

comparison of peak bili in physiologic jaundice between term and preterm

A

peak in term: 3-5 days
peak in preterm: 5 days

111
Q

risk of physiologic jaundice

A

1/200

112
Q

what is configurational isomerization

A

isomer changes form 4Z, 15Z to 4Z,15E which is less toxic
bilirubin –> bile

113
Q

what is structural isomerizations

A

the formation of lumirubin

114
Q

phototherapy dose depends on:

A
  1. spectrum: blue 460-490
  2. irradiation: 10uW/cm2/cm standard; 30 intensive
  3. exposure
  4. distance: optimal = 10-15 cm

SIDE

115
Q

when doing exchange transfusion what should the hct of replacement blood be?

A

50-55

116
Q

how many aliquots of blood for exchange

A

5-20

117
Q

how much of the blood volume is replaced?

A

87%

118
Q

how does ivig help jaundice

A

bind Fc receptor on reticuloendothelial cells so destruction of RBCs does not occur

119
Q

how does phenobarbital help jaundice

A
  • increase ligandin concentration, increasing uptake
  • inducing enzymes
  • increasing bile flow
120
Q

how does agar help jaundice

A

decrease enterohepatic circulation

121
Q

how does metalloporphyrins help jaundice

A

inhibit heme oxygenase

122
Q

bilirubin pathway

A

hemoglobin (hemeoxygenase)
heme + goblin
biliverdin (biliverdin reductase)
bilirubin [CO –> carboxyHgb] (glucuronyltransferases)
bilirubin mono and diglucuronides (glucuronidases)
stercobilinogen + urobilinogen

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