quiz

Question 1

Fanconi

Answer 1

DNA repair and damage, 12 different genes

Question 2

Shwachman Diamond

Answer 2

same gene, ribosomal RNA, mitotic spindle

Question 3

dyskeratosis

Answer 3

telomerase function abnormally short, Xlinked

Question 4

congenital amegakaryocytic thrombocytopenia

Answer 4

thrombocytopenia and absent kegakaryocytes, mutations in MPL

Question 5

paroxysmal nocturnal hemoglobinuria

Answer 5

hemolysis and BM failure, less GPI anchor on RBC, complement lysis, thrombosis

Question 6

toxic granulation

Answer 6

dark granules in neutrophils seen with infection or G-CSF

Question 7

Dohle bodies

Answer 7

ribosome rich ER seen in sepsis, G-CSF use

Question 8

Pelger-Huet

Answer 8

bilobed nucleus, myelodysplastic syndrome

Question 9

anemia

Answer 9

decrease in hemoglobin and hemotocrit

Question 10

MCV

Answer 10

size of RBC

Question 11

MCH

Answer 11

hemoglobin concentration

Question 12

MCHC

Answer 12

hemoglobin concentration in volume of red cells

Question 13

RDW

Answer 13

red cell distribution

Question 14

reticulocyte

Answer 14

pre red cell

Question 15

retic count

Answer 15

percentage of retics in RBC

Question 16

reticulocyte index

Answer 16

retic count X Hct / 40

should be greater than 2 in anemic patient with good response

Question 17

hypo vs hyper proliferative depends on…

Answer 17

reticulocyte index or absolute retic count

Question 18

tranferrin

Answer 18

iron transport in blood

Question 19

hepcidin

Answer 19

prevents iron release, prevents absorption, prevents iron export

Question 20

serum ferritin

Answer 20

relfect iron stores

Question 21

labs in iron def

Answer 21

low MCV
low MCH
decreased RBC
low retic

Question 22

serum iron studies in Fe def

Answer 22

low serum Fe
High TIBC - transferrin can bind
Ferritin - low
transferritin sat - low

Question 23

difference in anemia of chronic disease in iron test

Answer 23

decreased TIBC and increased or normal ferritin

Question 24

hereditary spherocytosis

Answer 24

defects in spectrin, ankyrin, band 3 and pallidin, memebrane deformability

Question 25

labs in HS

Answer 25

reticulocytes, spherocytes, osmotic fragility test, increase MCHC

Question 26

things seen in G6PD

Answer 26

Heinz bodies, ghost cells and bite cells

Question 27

warm vs cold AIHA

Answer 27

warm - IgG

cold IgM