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Neo Pathophys 2 > Anemia & Thrombocytopenia > Flashcards

Anemia & Thrombocytopenia Flashcards

1
Q

Describe the progression of production sites of Fetal erythropoesis (3)

A
  1. Yolk Sac (2-10wks)
  2. Liver (5wks-5-6 mos)
  3. Bone Marrow (18wk start-complete by 30 wks)
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2
Q

What would be the primary erythropoesis production site in a 24 wkr?

A

Liver

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3
Q

The hypoxic state in Utero leads to what?

What happens after birth?

A

Increased NRBC’s and Increased Reticulocytes.

W/in 72 hrs, NRBC’s disappear. By 7 days Reticulocytes <1%.

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4
Q

True/False. The umbilical vein stays patent long after the umbilical arteries constrict.

A

True.

Reason: to allow placental transfusion

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5
Q

How much fetal blood does the placenta contain?

A

~100 mL’s

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6
Q

By 1 min delayed cord clamping, how much fetal blood goes to the neonate?

A

50%

w/in 15 seconds-25%

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7
Q

Term infant has __- __ mL’s/kg blood volume.

Preterm infant has ___-___mL’s/kg blood volume.

A

50-100mL/kg

89-105mL/kg (higher plasma vol. RBC mass same as term)

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8
Q

What is normal Hgb range?

A

14-20g/dL

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9
Q

What is normal Crt range?

A

~42-60%

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10
Q

Hgb x ___ = Crt

A

x 3

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11
Q

Fetal–>Maternal transfusion can be caused by:

A

Amniocentesis

Trauma

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12
Q

How is fetal–>Maternal transfusion diagnosed?

A

Kleinhauer-Betke test
(detection of fetal Hgb on RBC’s in maternal blood)
Detects both the presence of and the volume of fetal RBC’s.
also flow cytometry avail-but more $$, not used much but more accurate

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13
Q

What is a significant % on KB test?

A

> 1% of maternal blood volume (or >50mL’s fetal blood)

-ie. if KB 2%, presume fetal transfusion 100mL’s.

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14
Q

When is a KB not useful/valid?

A

If mom also has a hemaglobinopathy with increased Hgb F

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15
Q

With Monochorionic/Monoamniotic twins, why would an OB choose to deliver them at 30-32 wks?

A

The longer the gestation, the higher the rate of intrauterine mortality. ~70% twin-twin transfusion.

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16
Q

Name 2 interventions in mono/mono twins to alleviate twin-twin transfusion.

A
  1. Serial Amnioreductions

2. Ablation (better overall survival rates)

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17
Q

Name S/S of twin-twin transfusion

A
  1. one bright pink baby, one very pale baby
  2. > 20% difference in BW
  3. > 5g Hgb difference is suspect (not dx)
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18
Q

The “donor” twin will have:

A
  1. Anemia

2. Oligohydramnios

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19
Q

The “recipient” twin will have:

A
  1. Polycythemia

2. Polyhydramnios

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20
Q

Would you want to give lots of blood to the donor twin quickly?

A

No, they are used to anemic state, you could cause them to be compromised. Replace very slowly.

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21
Q

How might the recipient twin need to be helped?

A

Elective exchange transfusion if high Crt to get it 70-75%.

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22
Q

Which twin is at more risk of cardiovascular and end-organ failure?

A

Recipient twin. Used to pumping high volume of sluggy blood.

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23
Q

Name 4 types of Hemorrhagic Anemia

A
  1. Fetal Hemorrhage
  2. Placental Hemorrhage
  3. Umbilical Cord Bleeding
  4. Hemorrhage r/t delivery
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24
Q

Name 4 reasons for umbilical cord bleeding

A
  1. Preemie (weak cord/rupture)
  2. Precip delivery (Increased cord tension)
  3. Short/entangled cord
  4. Abnormal cord insertion or vessels
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25
Q

Name 6 types of hemorrhage r/t delivery

-may be asymmptomatic first 24-48 hrs

A
  1. Cephalohematoma (no cross suture lines, restricted by periosteum)
  2. Subgaleal Hemorrhage (vacuum)
  3. ICH
  4. Adrenal/kidney H. (breech)
  5. Splenic rupture (w/splenomegaly)
  6. Hepatic hemorrhages
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26
Q

Name 3 reasons for hemolytic anemia

A
  1. Immune D/O’s (ABO/Rh)
  2. Acquired RBC D/O’s (infection)
  3. Hereditary RBC D/O’s (G6PD, Thalassemia)
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27
Q

What is ABO incompatability?

A

Result of maternal anti-A or anti-B antibodies that enter Fetal circulation and react with A or B antigens on Erythrocyte surface. (bABy + mOm = ABO)

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28
Q

Why don’t mothers with Blood types A, B, or AB tend to have ABO incompatabilities?

A

They tend to produce IgM (which doesn’t cross placenta)

Type O blood produces IgG-which does cross.

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29
Q

Do you have to be sensitized to have ABO?

A

No, can occur with 1st pregnancy.

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30
Q

Is ABO common? Is it usually serious?

A

Yes, common -12% pregnancies affected.

No, <1% live births w/serious hemolysis. Only a small fraction of the anti-A or Anti-B binds to the erythrocytes.

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31
Q

W/ABO, is DAT positive?

Is Indirect antiglobulin test positive?

A

DAT usually positive, can be negative.

Indirect Antiglobulin test positive.

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32
Q

When should a baby w/ABO be checked for anemia post-discharge?

A

2-3 wks

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33
Q

What is the other name for Rh incompatibility?

A

Erythroblastosis Fetalis

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34
Q

When does Rh incompatibility happen?

A

Rh+ mom (RBC w/D Antigen)

& Rh- baby (RBC w/Anti-D Antibodies)

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35
Q

Is sensitization necessary for Rh incompatability?

What is the incidence of Rh incompatibility after Rhogam came out?

A

No, can happen first known pregnancy (hx of unknown miscarriage, transfusion)
Increased incidence w/subsequent pregnancies.

11/10,000

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36
Q

Can fetuses inherit Rh +/- from mom or dad?

A

Yes. They can either match mom or dad’s blood typing.

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37
Q

When is Rhogam given?

A

1st at 28 wks or w/trauma/fall, etc

Then at birth (if infant is Rh+)

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38
Q

What is the cause of the varying degrees of Rh dz (jaundice-death)?

A

The degree of Anemia from hemolysis.

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39
Q

Name the 3 types of Hemolysis

A
  1. Mild
  2. Moderate
  3. Severe
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40
Q

Which type of Hemolysis is most common?

A

Mild

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41
Q

Describe Mild Hemolysis

A

Hgb >14g/dL; Cord bili <4g/dL
Positive DAT w/minimal hemolysis
Jaundice-tx w/phototherapy usually

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42
Q

Describe Moderate Hemolysis

A

Hgb <14g/dL; Cord bili >4g/dL
Moderate Anemia w/hemolysis
Hepatosplenomegaly, jaundice
Tx: early exchange & intensive photo. tx

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43
Q

What is the incidence of severe hemolysis?

Describe Severe Hemolysis.

A

~25%-of affected infants
Hgb <14g/dL; cord bili >14g/dL
Moderate Anemia w/hemolysis
Hydrops as early as 20-22 wks
Early detection via MCA doppler at 24 wks
Tx: Amniocentesis, early induction, intrauterine fetal blood transfusions, photo tx, exchange, IVIG PRN

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44
Q

In severe hemolysis, why are intrauterine fetal blood transfusions done?

A

To prevent hydrops

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45
Q

How is a minor blood incompatibility Dx’d?

A

DAT+ in absence of Rh and ABO incompatibility w/Neg Maternal DAT

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46
Q

How common is Kell sensitization?

A

Fairly common, 20%

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47
Q

Name the order of minor blood group incompatabilities.

A 
D
c,E (Rh Antigens)
Kell (K, k)
Duffy
Kidd
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48
Q

If a mom has anti-Kell, be prepared for what?

A

A baby w/Severe hemolysis at delivery (regardless of what US says)
*Amnio and antibody titer may underestimate hemolysis severity

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49
Q

Name an autoimmune dz that can cause anemia.

What happens?

A

Lupus

Passive transfer of IgG antibody

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50
Q

When would you suspect Auto-immune anemia?

A

Neonatal hemolysis
+DAT (Absence of Rh or ABO incompatibility)
Antiglobulin + hemolysis in mother

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51
Q

How do you tx mom/baby w/auto-immune anemia?

A

Mom: Prednisone
Baby: Exchange, Phototherapy

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52
Q

Infection can cause non-immune hemolytic dz. From what infections?

A

CMV, Toxoplasmosis, Syphilis, bacterial sepsis

*can also have thrombocytopenia

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53
Q

What signs would you see on non-immune hemolytic dz?

How would you tx?

A

Jaundice (T & C elevated), Hepatosplenomegaly

Supportive tx

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54
Q

Is hemolysis r/t infection always early?

A

No, may be weeks later

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55
Q

Name the 2 dominantly inherited abnormal RBC morphology d/o’s

A
  1. Spherocytosis

2. Eliptocytosis

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56
Q

How is Spherocytosis dx’d?

When is it comfirmed?

A 
Blood smear (although can also be seen w/ABO-so r/o)
After 3 months (when HgbF is gone)
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57
Q

What happens in Eliptocytosis?

A

Mutations of the RBC membrane that weaken it’s structure–>cell destruction.

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58
Q

Name the 2 RBC enzyme abnormalities

A
  1. G6PD

2. PK

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59
Q

In general, what is G6PD?

A

An enzyme deficiency.
Sex-linked, X-chrom.
Males>females affected
Mediterranean, African, Middle-Eastern, Asian descent
*provides measure of protection from Malaria (US=increase hyperbili, anemia)

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60
Q

What is PK deficiency?

A

Autosomal Recessive
Affects all ethnic groups
Rare compared to G6PD

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61
Q

What is the pathophys of G6PD?

A

RBC’s lack enzyme to regenerate GSH–>oxidative injury–>Heinz bodies bind to denatured cell membrane–>hemolysis

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62
Q

In what situation might you suspect G6PD?

A

DAT- hemolytic anemia w/infection or admin. of drugs

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63
Q

How can G6PD be dx’d?

A

Peripheral blood smear

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64
Q

What is the major manifestation of G6PD?

A

Hyperbilirubinemia (rarely present at birth)

Jaundice > Anemia (prolonged hyperbilirubinemia)

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65
Q

> ___% kenicterus cases are from G6PD?

A

> 30%

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66
Q

Is G6PD on all NBMS?

A

No. Being pushed though.

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67
Q

The term infant has about ___-___% HgbF

A

60-90%

68
Q

HgbF % falls to ____ by 6 months age?

A

~5%

69
Q

What is postnatal Hgb?

A

HgbA

70
Q

HgbA has how many alpha/beta chains?

A

2 Alpha, 2 Beta

71
Q

Name the 2 major causes of RBC Aplasia.

Are these common in NICU?

A
  1. Diamond-Blackfan Anemia (congenital hypoplastic anemia)—severe Anemia in NB period
  2. Transient Erythroblastopenia of Childhood (TEC)—rare before 6 mos
72
Q

Describe Physiologic Anemia

A

Increased O2 content decreases Erytrhopoetin–>decreased Erythropoesis

73
Q

When and what is the Nadir in a term infant?

A

Wks 6-12

Hgb 9.5-11 g/dL

74
Q

When and what is the Nadir in a preterm infant?

A

Wks 5-10

Hgb 8-10 g/dL

75
Q

When does RBC begin to increase?

A

When tissue O2 demand exceeds supply, Hgb is produced

76
Q

What are the symptoms of Physiologic Anemia?

A

Asymmptomatic

77
Q

Does Fe admin prevent Physiologic Anemia?

A

No, only stores Fe for future use.

78
Q

Name 4 causes of Anemia of Prematurity

A
  1. Physiologic decrease in Hgb
  2. Heightened deficiencies of Folate, Vit B12, Vit E-essential in erythrocyte integrity
  3. Iatrogenic blood loss
  4. Underlying Dz. (Rh, ABO)
79
Q

Name the tx’s for Apnea of Prematurity

A
  1. Limit lab testing
  2. Delayed cord clamping (30 sec preemie)
  3. Optimize nutrition
  4. Iron Supplement
  5. EPO
  6. PRBC transfusions
80
Q

What is the calculation for when a premature infant will deplete his iron stores?

A

1.5 x cord Hgb level = + % of BW needed

81
Q

True/False: Fe deficiency has negative neurodevelopmental sequelae.

A

True

82
Q

What are the 2 types of Fe supplement we use?

A
  1. Fer-in-sol
  2. Poly-vi-sol
    (usually transitioned to poly in prep for home)
83
Q

How much Fe do most formulas provide?

A

2mg/kg/day

84
Q

How much Fe do you want a preemie to get?

A

2-6 mg/kg/day (feeds + fe supp)

85
Q

What is the Fe concentration in Poly-vi-sol?

A

10 mg/kg

86
Q

What is the usual transfusion amount/kg?

A

10-20 mL’s/kg

87
Q

3 mL’s/kg transfusion will raise Hct by ~?

10 mL’s/kg transfusion will raise Hgb by ~?

A

about 3% (would increase Hgb by 1 g/dL)

about 3 g/dL (would increase Crt by 10%)

88
Q

Name the 5 risks of blood transfusion

A
  1. Graft vs Host
  2. Infection (CMV)
  3. Suppress own RBC production
  4. Transfusion associated NEC
  5. Fluid overload (s/s pulm. edema)
89
Q

Irradiation of transfusion products reduces what?

A

CMV and Graft/host dz

90
Q

Leukocyte reduction of transfusion products reduces what?

A

CMV infection

91
Q

Is there concrete guidelines for stopping feedings with/before transfusions r/t NEC?

A

No, great variance. Studies suggest it’s the Anemia not the transfusion causing the NEC.

92
Q

What number Defines Thrombocytopenia

A

Platelet count <150

93
Q

What number defines Mild thrombocytopenia?

A

Platelet count 100-150

94
Q

What number defines Moderate Thrombocytopenia?

A

Platelet count 50-99

95
Q

What number defines Severe Thrombocytopenia?

A

Platelet count <50

96
Q

Where do platelets come from?

A

Megakaryocytes in the bone marrow

97
Q

Who has a greater chance of developing thrombocytopenia, <800 gms or >800 gms?

A

<800 gms –85%

98
Q

Is congenital thrombocytopenia rare?

A

Yes

99
Q

True/False: Immune acquired thrombocytopenias are not Uncommon

A

True

100
Q

Name the signs you may see in a baby with Thrombocytopenia

A
  1. Bruising
  2. Purpura or Petechiae
  3. Bleeding from needle sticks
  4. Oozing from line sites
  5. Asymmptomatic
101
Q

How is early-onset Thrombocytopenia defined?

A

< 72 hrs

102
Q

W/early-onset thrombocytopenia in an ill-appearing infant, what are the 3 differentials?

A

TORCH infection
Birth Asphyxia
Sepsis

103
Q

W/early-onset thrombocytopenia in a well-appearing infant, what are the 3 differentials? (mild-mod, & severe)

A

Mild-mod: Placental insufficiency, genetic D/O’s, Autoimmune

Severe: Neonatal Alloimmune Thrombocytopenia, Genetic D/O’s

104
Q

How is late-onset thrombocytopenia defined?

A

> 72 hrs

105
Q

What is the differential in an ill-appearing infant with late-onset thrombocytopenia?

A

Sepsis, NEC, inborn error of metabolism

106
Q

What is the differential in a well-appearing infant with late-onset thrombocytopenia?

A

Drug-induced thrombocytopenia
thrombosis
Fanconi-Anemia (inherited, affects all types of blood cells)

107
Q

What is NAIT?

A

Neonatal Alloimmune Thrombocytopenia

108
Q

When should it be considered?

A

Any infant with initial Platelet count <50 in well-appearing infant.

109
Q

What happens in NAIT?

A

Passive transfer of maternal Allo-antibodies attach paternally-derived platelet antigens that are present on the neonate’s platelets.

110
Q

When an infant has NAIT, is does mom have Thrombocytopenia?

A

No, her Plt ct is normal.

111
Q

How can NAIT be tested for?

A

Maternal and Paternal blood samples; Antigen testing HPA 1, 3, 5 (catches most cases)
*The baby can be screened too, but high false-positive rate.

112
Q

How is NAIT tx’d?

A
  1. Cranial US (25% ICH-often in utero)
  2. Random donor Plt transfusion <30 or <100 w/IVH
  3. IVIG if dx confirmed
  4. Corticosteroids
  5. If other options have not increased plt in 1-2 days: Matched (Antigen Neg platelets): either maternal OR Donor HPA matched.
113
Q

If a platelet transfusion of 5-10mg/kg is given, how much would you expect the platelet count to increase?

A

50,000-100,000

114
Q

What does ITP stand for?

A

Immune Thrombocytopenic Purpura

115
Q

What happens/why does baby get it?

A

Mom has Auto-Immune Thrombocytopenia or is developing it during pregnancy.
*Maternal Autoantibodies are passively transferred to fetus in utero which react with it’s platelets.

116
Q

In OB pts with ITP, ___% of the infants had low platelet count at birth?

A

25%

117
Q

What condition might mask maternal and neonatal thrombocytopenia?

A

HELLP

118
Q

How is ITP tx’d?

A
  1. Screen infants born to mothers w/autoimmune dz’s
  2. Cranial US
  3. Normal Plt count: Observe them
  4. Mild to Mod (50-100k): Trend down < 30, tx w/IVIG
  5. Severe (< 50k): < 30 tx w/IVIG (first line); transfuse w/random donor platelet transfusion
119
Q

What is the normal Platelet transfusion amount?

A

10-15 mL’s/kg aliquot

120
Q

When is Platelet transfusion recommended for infant < 33wks in the first WOL?
After the first WOL (if clinically stable)?

A

<50,000

<30,000

121
Q

What is transfusion associated lung injury?

When does it show up?

A

Hypoxia and bilateral pulmonary lung infiltrates.

W/in first 6 hrs of transfusion.

122
Q

There’s a strong A/W ________and Platelet transfusion in NICU population.

A

Mortality

chicken/egg concept

123
Q

Name 4 reasons Neonates have excessive bleeding

A
  1. Platelet D/O’s
  2. Neonatal hemophilia or clotting factor D/O’s
  3. Vit K deficiency syndroms
  4. DIC
124
Q

What is a normal PT?
Is it an extrinsic or intrinsic pathway?
What does it affect?
PT is elevated in?

A

10-16 sec (Prothrombin Time)
Extrinsic
Vit K dependent factors
Liver Dz, and DIC

125
Q

Which pathway is PTT?

When would you see an elevated PTT?

A

Term= 25-60 sec, Preemie= up to 80 sec.
(Partial Thromboplastin Time)
Vit K deficiency, Liver failure, DIC, factor deficiencies

126
Q

What factor is Fibrinogen?

A

Factor 1

127
Q

What is a D-dimer?

A

Firbrin degradation products (fibrin split products)

128
Q

What does INR stand for?
If it is high, what is more likely to happen to baby?
If it is low, what is more likely to happen to baby?

A

International Normalized Ratio
High-Bleed (just more likely to, does not indicate active bleeding)
Low-Clot

129
Q

Name the 6 screening tests for coagulation

A 
PT
PTT
Fibrinogen
D-Dimer
Platelets
INR
130
Q

What is Von Willebrand Factor?

A

Primary plasma protein required for platelet adhesion/aggregation

131
Q

What happens in Von Willebrand Dz?

A

Absent or decreased amounts of OR Abnormal VWF function–>defects in platelet adhesion/aggregation–>increased bleeding risk

132
Q

VWF is the carrier protein for which factor?

A

Factor 8

133
Q

How does VWF present in NB period?

A

Rarely presents itself in NB period, s/s similar to Thrombocytopenia (bleeding/bruising)

134
Q

How is VWD treated?

A

Replace VWF concentrates from plasma (FFP).

Desmopressin or antifibrinolytic agents (rarely used in NB’s)

135
Q

Factor 8 is known as?

Factor 9 is known as?

A

Hemophilia A

Hemophilia B

136
Q

How is Hemophilia transmitted?

A

X-linked. Males>Females

w/known family Hx, can be Dx’d before symptoms.

137
Q

What happens in Hemophilia?

A

Absence of Factor 8 & 9–>Reduced Thrombin on Plts at site of bleeding–>Clot w/poor structural integrity–>Increased risk Fibrinolysis

138
Q

What is the incidence of severe Hemophilia A (Factor 8 deficiency)?

What is the incidence of Hemophlia B (Factor 9 deficiency)?

A

1: 10,000
1: 30,000

139
Q

When can Hemophilia manifest itself in the NB period?

A

With Severe Hemophilia w/Factor level <1%

140
Q

Name Clinical Manifestations of severe hemophilia

A

Intra or extracranial bleeding
Prolonged bldg from venipuncture/heelstick
Prolonged bldg after circ (most common in asymptomatic infants)
Excessive bruising
Muscle hematomas after IM’s like vaccines
Bleeding after surgery

141
Q

How is Severe Hemophilia tx’d?

A

Factor replacement Tx

142
Q

What labs are used to Dx severe hemophilia? Does this work in a preemie?

A

Elevated PTT, abnormal factor levels

Misleading in preemie, deficient in these anyway.

143
Q

What causes Vit K deficiency in a NB?

A

Insufficient intestinal colonization by bacteria, poor absorption

Low Vit K in breastmilk

144
Q

What is the most common cause of bleeding in an otherwise healthy infant?

A

Vit K deficiency

145
Q

Infants who do not recieve Vit K at birth are ____ x’s more likely to develop late VDKB

A

81 x’s >

146
Q

What lab is elevated w/VKDB?

A

3-4 fold PT (is specfic to Vit K)–the PT is greater than PTT–

147
Q

Early VKDB happens?

Can be from?

A

w/in 24 hrs

Poor maternal Vit K transfer, maternal meds

148
Q

Classic VKDB happens?

From?

A

1-7 days

Physiologic deficiency, lack Vit k in breastmilk or inadequate feeding

149
Q

Late VKDB happens?

From?

A 
2-12 wks
Inadequate VitK intake (usually exclusively breastfed)
Fat malabsoption (biliary atresia, CF, Alpha 1 anti-trypsin)
150
Q

How do you tx VKD?

How long does the measure last?

A

0.5-1mg Vit K IM at birth

1 month

151
Q

DIC is a ___________ coagulopathy defined as?

A

Consumptive: Uncontrolled activation and consumption of Platelets, Pro-coagulant, Anti-coagulant, Fibrinolytic proteins.

152
Q

What are the most common causes of DIC?

How do they present?

A

Sepsis, NEC (each present with thrombocytopenia initially and most prominently)
Hypoxia/Acidosis (present with Fibrinogen deficiency)
Liver failure

153
Q

How is successful treatment of DIC defined?

A

Improvement of coagulation w/in 72 hrs, and resolution of thrombocytopenia in 7-10 days

154
Q

What symptom is the first to show and last to leave with DIC?

A

Thrombocytopenia

155
Q

What are the indications for using FFP?

A

Bleeding, DIC, Vit K deficiency, Factor 8 Deficiency, Factor 9 deficiency

156
Q

What are 2 main factors in FFP?

A

Factor 5 and factor 8

157
Q

What are the indications for Cryoprecipitate?

A

Factor 8 deficiency, VWF deficiency,

158
Q

What are the components of Cryoprecipitate?

A

Fibrinogen, Factor 8, Factor 9, VWF, Fibronectin

159
Q

What are the indications for Platelets?

A

Thrombocytopenia, Bleeding

160
Q

What are the components of Platelets?

A

Platelets +/- some WBC’s (how graft vs host can happen)

161
Q

What is the pathophys of Thrombosis in NB?

A

Hypercoagulability, Disturbances in blood flow, Endothelial damage/disruption

162
Q

Why are infants at greater risk of Thrombosis vs. older children?

A

Decreased fibrinolysis d/t low plasminogen levels

163
Q

What can put baby at risk of Thrombosis?

What deficiencies can put baby at risk of Thrombosis?

A

Umbilical lines (site of insertion or tip), Asphyxia, Sepsis (w/DIC), Polycythemia (Dehydration, IDM, CHD), Shock

Protein C or S deficiency, ATIII deficiency, Factor V Leiden

164
Q

What are some clinical types of Thrombosis?

A

Renal vein thrombosis, renal artery thrombosis, sagittal sinus thrombosis, stroke (seizures)

165
Q

How is Thrombosis Dx’d?

A

US w/doppler, CT, MRI

166
Q

How is Thrombosis Tx’d?

A

Anticoagulant Tx:
Heparin infusion
Low-molecular-weight heparin (enoxaprin SQ
Warfarin (PO)

Thrombolysis if complete occlusion or ischemia:
Systemic TPA

Neo Pathophys 2 (20 decks)

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