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Neo Boards GF > Heme/Onc/Bili > Flashcards

Heme/Onc/Bili Flashcards

1
Q

What is first site of hematopoiesis? Second? Third?

A
  1. Secondary yolk sac (until 6 weeks)
  2. Fetal liver (6-22 weeks)
  3. Bone marrow (primary 22+, starts 8-19)
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2
Q

How do RBC indices change as GA increases? Hct? MCV? RBC number? Retic? nRBC?

A

Increases: hct, RBC number
Decreases: MCV, nRBC
*Retic peaks at 26-27 wks then declines

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3
Q

How much fetal hemoglobin do term infants have? At 6 months of age?

A

Term - 80%

6 months - 5%

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4
Q

What chromosomes are alpha and beta globin genes on?

A

Alpha - chr 16

Beta - chr 11

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5
Q

What makes up HgbF?

A

2 alpha and 2 gamma

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6
Q

What makes up Hgb A? A2?

A 
A = 2 alpha, 2 beta (inc quickly after birth)
A2 = 2 alpha, 2 delta (inc tiny bit steadily after birth)
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7
Q

What are signs of various alpha thal?

A

1 missing - silent carrier (all normal)
2 missing - trait, normal to inc Hb Barts (4-6%), may be asymptomatic or mild microcytosis
3 missing - Hb H (4 beta chains), infant has 20-30% Barts, child with 4-20% Hb H, moderate severe anemia, Heinz bodies. May need splenectomy
4 missing - no Hb A or F, almost all Bart’s. Hydrops, rare, very severe

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8
Q

What is Hb Gower and when is it expressed?

A 
2 zeta (or alpha) chains and 2 epsilon chains
Embryonic from the yolk sac
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9
Q

Where is Hb F made?

A

Fetal liver

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10
Q

What is inheritance of HbSS? What is mutation?

A

Autosomal recessive

Valine (instead of glutamic acid) in beta globin gene

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11
Q

What will sickle cell patients be missing?

A

HbA (no beta chains)

Presents ~6 months

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12
Q

What is Hemoglobin E?

A

Most common worldwide hemoglobinopathy
Decreased transcription beta gene
Mild chronic microcytic anemia

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13
Q

How many pregnancies have fetal maternal hemorrhage?

A

50-75% (most very small amount)
1/400: >30 ml
1/2000: >100 ml

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14
Q

What is Kleihauer Betke calc ?

A

% Hb F = fetal cells/ maternal cells x 100

1% = 50 ml fetal blood

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15
Q

What are signs of Diamond-Blackfan anemia?

A

Pure red cell aplasia
Macrocytic anemia, low retic. High epo. Bone marrow with absent erythroid precursor
Also w abnormal face, 3 joint thumbs, short, heart/renal anomalies
Need chronic transfusion, BMT

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16
Q

What is Fanconi’s anemia?

A

Constitutional aplastic anemia
Autosomal recessive
Chromosome instability (test w mitomycin C -> lots of breaks)
Pancytopenia after 5 years
Also short, abnl thumbs, small head, hyperpigmented

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17
Q

What are signs of vit B12 and folate deficiency?

A

Hypersegmented neutrophils

Macrocytic anemia, low retic

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18
Q

What causes microcytic anemia?

A

Thalassemia
Iron deficiency
Lead poisoning
Infection

19
Q

What causes Macrocytic anemia?

A 
Methylmalonic aciduria 
Folate/B12 deficiency
Acquired aplastic anemia
Fanconi anemia
Diamond Blackfan anemia
Drugs
Hypothyroid
20
Q

When is physiologic nadir in term infants? Preterm?

A

Term - 10-12 wks (Hb 9)

Preterm - 8 weeks or earlier (Hb 7-8)

21
Q

What is difference between direct Coombs and indirect Coombs?

A

Direct: detects antibodies on surface of RBCs
Indirect: detects antibodies in serum (mixes serum with RBCs having known antigen to see if antibodies stick)

22
Q

How is hereditary spherocytosis inherited? Diagnoses?

A

Most autosomal dominant

Test: osmotic fragility test (expose RBCs to hypo-osmolar solutions) -> will lyse

23
Q

What is inheritance of G6PD? Findings?

A

X linked recessive

Heinz bodies, shorter RBC lifespan, anemia

24
Q

How to diagnose G6PD?

A

Test for enzyme activity (but young RBCs have higher levels so don’t test during crisis bc most will be young RBCs)

25
Q

How is pyruvate kinase deficiency inherited?

A

Autosomal recessive (2nd most common inherited RBC enzyme defect, 2nd to G6PD)
Disruptions of Embden-Myerhoff pathway and pentose phosphate shunt
Signs: chronic anemia (normocytic) high 2,3 DPG

26
Q

What is the formula for partial volume exchange?

A

Blood vol exchanged = [(obs hct - desired hct)/obs hct ] x infants blood vol

27
Q

What is normal level of met hemoglobin?

A

3%

28
Q

What are signs of methemoglobinemia?

A

Normal paO2 but low sat
Brown blood when exposed to air
Hb is oxidized in ferric state so can’t bind O2

29
Q

What makes platelets? When does production start?

A

Megakaryocytes

8 weeks GA

30
Q

What is most common antigen in NAIT?

A

HPA-1a (80-90% of cases)

31
Q

What do you expect of plts in Thrombocytopenia absent radii syndrome (TAR)?

A

Severe low plts at birth, with absent or reduced megakaryocytes
If survive past 4 months, gradual resolution of thrombocytopenia by school age

32
Q

What is amegakaryocyte thrombocytopenia?

A 
Rare, x linked condition
Severe isolated thrombocytopenia 
No other abnormalities
50% develop aplastic anemia
High mortality, risk of leukemia
33
Q

Do maternal clotting factors cross placenta?

A

No, all clotting factors are from fetus

34
Q

When do clotting factors reach adult levels?

A

6 months of age

35
Q

What is hemophilia A?

A 
Factor VIII deficiency
X linked recessive
70% of all hemophilia 
Severe (<1% factor level)
Mod (1-5%)
Mild (>5%)
36
Q

What is hemophilia B?

A

Factor IX deficiency
X linked recessive
30% of all hemophilia

37
Q

What is hemophilia C?

A 
Factor XI deficiency
Autosomal recessive
Associated w Noonan
Doesn’t matter how much factor, no correlation with severity
Treat with FFP
38
Q

What is factor XIII deficiency?

A

Autosomal recessive
Normal clotting studies but bleeding from umbilical stump or several days after circ
Treat with cryo or factor XIII concentrate

39
Q

What is von Willebrand disease?

A

Autosomal dominant or recessive
Component of factor VIII that attaches platelet to vessel
Rarely presents in newborns
Abnl bleeding time, usually normal PT and plts.
Dx: check ristocetin factor

40
Q

What are neonatal risks for vitamin K deficiency?

A 
Maternal anticonvulsant meds
Liver disease
Malabsorption 
Antibiotics
Coumadin
Breastfeeding
41
Q

How do you diagnoses vit K?

A

Isolated prolonged PT! (Only have prolonged PTT if deficient for extended time)

42
Q

What is most common solid tumor in neonatal period? Second most common?

A
  1. Teratoma

2. Neuroblastoma

43
Q

How much blood volume is replaced in a double volume exchange?

A

87%

44
Q

What is primary way phototherapy reduces bilirubin?

A

Structural isomerization to lumirubin which can be excreted in bile or urine

Neo Boards GF (14 decks)

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