Hematology Flashcards
What disorders do not have elevated PT
Hemophilia, and Von Willebrand
2-3 DPG reduces Hgb affinity, how does it do this?
bohr effect, reduces intra-ethrocyte ph (indirectly)
A 10 ml/kg transfusion of PRBC raises hgb how much?
2 to 3 g/dL
Bleeding at umblical cord site few weeks after separation. coags and platelet count normal
Factor XIII deficiency
deficiency in Vitamin K leads to increase in what clotting factor?
PT time, essenital in clotting factors 2, 7, 9, 10
Does MCV increase or decrease with gestational age?
decreases (almost other RBC indices increase)
G6PD deficiency leads to a lack of what?
lack of NADPH, which needing to maintain antioxidants within RBC
How can growth restriction cause polycythemia?
The newborn compensates for poor placental oxygen transfer with polycythemia that can then result in multiorgan thrombosis at or after birth.
how do you diagnose neonatal hereditary shperhocytosis?
MCHC/MCV ratio
MCHC significantly elevated and MCV depressed
How does Apt Test Work?
Add NaOh to solution, Fetal HB resistant to alkali denaturation
Fetal hgb is pink on slide
How is marked erthyrocytosis avoided in fetus?
Hepatic EPO is inensitive to tissue hypoxia (unlike renal)
How would you diagnose Vitamin K Deficiency?
Des-gama-carboxy prothrombrin, protein induced by Vitamin K absence
Order of fetal hematopoiesis (Sites)
fetal yolk sac (through 8 weeks)
fetal liver ( through 22 weeks)
bone marrow (after 22 weeks)
Pyruvate kinase deficiency leads to a lack of what?
leads to lack of ATP, leading to chronic anemia
What anatomical finding can cause impaired hepatic uptake of bilirubin?
patent or partially patent ductous venosous
What are electrolyte abnormalities you see in polycythemia?
hypocalcemia, and hypoglycemia
what can cause hydrops fetalis?
Rh- incompatability, chronic fetomaternal hemorrhage, homozogyous alpha thal, homozygous, G6PD
What causes RBC storage lesions
Changes in physical structure
Altered RBC metabolism
Depletion of Nitric Oxide
Humoral Factors
Why do you see more hemolysis with O blood type?
O type includes antibody of IgG class, which can cross placenta
What intervention should be avoided in Kasabach Merritt phenomenon
platelet transfusion (even though there is thrombocytopenia)
Platelets may become trapped within the lesion, leading to further platelet activation and worsening of KMP.
What is this
Howell Jolly Bodies
Inclusion of Nuclear Chromatin
When do you see this?
Target cells
Alpha thal, and after splenectomy
What blood product should you avoid in Kasabach Merrit?
even though they are thrombocytopenic, don’t give plateletes, they can become trapped
you can give Cyroprecipitate
UGT1A1
(UDP-glucuronosyltransferase 1A1)
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]
Acid Maltase deficiency seen in what?
Pompe Disease
What are CD4 Cells?
What is beta-glucuronidase
deconjugates bilirubiin, returning it to enterohepatic circulation
See this is breastmilk jaundice
