Hematology

Question 1

What disorders do not have elevated PT

Answer 1

Hemophilia, and Von Willebrand

Question 2

2-3 DPG reduces Hgb affinity, how does it do this?

Answer 2

bohr effect, reduces intra-ethrocyte ph (indirectly)

Question 3

A 10 ml/kg transfusion of PRBC raises hgb how much?

Answer 3

2 to 3 g/dL

Question 4

Bleeding at umblical cord site few weeks after separation. coags and platelet count normal

Answer 4

Factor XIII deficiency

Question 5

deficiency in Vitamin K leads to increase in what clotting factor?

Answer 5

PT time, essenital in clotting factors 2, 7, 9, 10

Question 6

Does MCV increase or decrease with gestational age?

Answer 6

decreases (almost other RBC indices increase)

Question 7

G6PD deficiency leads to a lack of what?

Answer 7

lack of NADPH, which needing to maintain antioxidants within RBC

Question 8

How can growth restriction cause polycythemia?

Answer 8

The newborn compensates for poor placental oxygen transfer with polycythemia that can then result in multiorgan thrombosis at or after birth.

Question 9

how do you diagnose neonatal hereditary shperhocytosis?

Answer 9

MCHC/MCV ratio
MCHC significantly elevated and MCV depressed

Question 10

How does Apt Test Work?

Answer 10

Add NaOh to solution, Fetal HB resistant to alkali denaturation
Fetal hgb is pink on slide

Question 11

How is marked erthyrocytosis avoided in fetus?

Answer 11

Hepatic EPO is inensitive to tissue hypoxia (unlike renal)

Question 12

How would you diagnose Vitamin K Deficiency?

Answer 12

Des-gama-carboxy prothrombrin, protein induced by Vitamin K absence

Question 13

Order of fetal hematopoiesis (Sites)

Answer 13

fetal yolk sac (through 8 weeks)
fetal liver ( through 22 weeks)
bone marrow (after 22 weeks)

Question 14

Pyruvate kinase deficiency leads to a lack of what?

Answer 14

leads to lack of ATP, leading to chronic anemia

Question 15

What anatomical finding can cause impaired hepatic uptake of bilirubin?

Answer 15

patent or partially patent ductous venosous

Question 16

What are electrolyte abnormalities you see in polycythemia?

Answer 16

hypocalcemia, and hypoglycemia

Question 17

what can cause hydrops fetalis?

Answer 17

Rh- incompatability, chronic fetomaternal hemorrhage, homozogyous alpha thal, homozygous, G6PD

Question 18

What causes RBC storage lesions

Answer 18

Changes in physical structure
Altered RBC metabolism
Depletion of Nitric Oxide
Humoral Factors

Question 19

Why do you see more hemolysis with O blood type?

Answer 19

O type includes antibody of IgG class, which can cross placenta

Question 20

What intervention should be avoided in Kasabach Merritt phenomenon

Answer 20

platelet transfusion (even though there is thrombocytopenia)
Platelets may become trapped within the lesion, leading to further platelet activation and worsening of KMP.

Question 21

What is this

Answer 21

Howell Jolly Bodies
Inclusion of Nuclear Chromatin

Question 22

When do you see this?

Answer 22

Target cells
Alpha thal, and after splenectomy

Question 23

What blood product should you avoid in Kasabach Merrit?

Answer 23

even though they are thrombocytopenic, don’t give plateletes, they can become trapped

you can give Cyroprecipitate

Question 24

UGT1A1
(UDP-glucuronosyltransferase 1A1)

Answer 24

Gene you see in Glibert, Crigler Najgar
don’t see any conjugated bili in Crigler Najagr

enzyme of the glucuronidation pathway that transforms small lipophilic (fat-soluble) molecules, such as steroids, bilirubin, hormones, and drugs, into water-soluble, excretable metabolites.[7]

Question 25

Acid Maltase deficiency seen in what?

Answer 25

Pompe Disease

Question 26

What are CD4 Cells?

Answer 26

Question 27

What is beta-glucuronidase

Answer 27

deconjugates bilirubiin, returning it to enterohepatic circulation
See this is breastmilk jaundice