Heme 4

Question 1

3 ways that cells break down proteins

Answer 1

ca dep
ubiquitin
lysosomal

Question 2

HIV Rx that causes BM suppression

Answer 2

zidovudine

Question 3

treatment for lead poisoning

Answer 3

adults

• succimer
• EDTA

children

• dimercaprol
• EDTA
• succimer

Question 4

Dx: child with anemia since birth, cure with splenectomy

Answer 4

hereditary spherocytosis

Question 5

complications of hereditary spherocytosis

Answer 5

anemia/jaundice

pigment stones

splenomegaly

coombs negative

Question 6

what is the coombs test

Answer 6

RBC agglutination with the addition of antihuman antibody

Question 7

Ig seen in cold agglutinins

Answer 7

IgM

Question 8

when do you see cold agglutinins

Answer 8

mycoplasma

EBV

malignancy

Question 9

Ig seen in warm agglutinins

Answer 9

IgG

Question 10

when do you see warm agglutinins

Answer 10

virus

SLE

malignancy

congenital immune abnormality

Question 11

what are schistocytes

Answer 11

helmet cells

seen in

• DIC
• TTP

Question 12

what protozoa can cause hemolytic anemia

Answer 12

babesia

malaria

Question 13

hematologic disorder a/w:

hams test

Answer 13

PNH

Question 14

hematologic disorder a/w:

DEB test

Answer 14

fanconis anemia

Question 15

hematologic disorder a/w:

heinz bodies

Answer 15

G6PD

Question 16

hematologic disorder a/w:

basophilic stippling

Answer 16

Thalassemia
AOCD
Iron def
lead poisoning

Question 17

hematologic disorder a/w:

osmotic fragility

Answer 17

spherocytosis

Question 18

Dx and Rx: 11 year old with chronic non healing ulcer on his foot, x ray shows calcified spleen

Answer 18

sickle cell

hydroxyurea

Question 19

what type of cells are associated with microangiopathic anemia

Answer 19

schistocytes

Question 20

what is a/w microangiopathic anemia

Answer 20

DIC

TTP

SLE

Malignant HTN

Question 21

what can cause macroangiopathic anemia

Answer 21

prosthetic valves

aortic stenosis

Question 22

when would you usually see a coombs positive test

Answer 22

autoimmune hemolysis

Question 23

how can you differentiate intravascular from extravascular hemolysis

Answer 23

intra

• decreased haptoglobin
• no splenomegaly

extra

• increased UCB
• splenomegaly

Question 24

Rx for spherocytosis

Answer 24

splenectomy

Question 25

what virus can lead to aplastic crisis

Answer 25

parvo B19

Question 26

what causes the damage in G6PD def

Answer 26

failure of HMP shunt under oxidative stress

Question 27

what makes G6PD def worse

Answer 27

sulfa drugs

infection

fava beans

Question 28

what cells are a/w G6PD def

Answer 28

heinz bodies

bite cells

Question 29

a/w hemolytic anemia in a new born

Answer 29

pyruvate kinase def

Question 30

what causes the damage in pyruvate kinase def

Answer 30

dec ATP

no Na/K ATPase
-cell swelling and lysis

Question 31

what can make sickle cell worse

Answer 31

low O2

dehydration

low pH

Question 32

what is the defect in sickle cell

Answer 32

substitution on glutamate for valine

defect in protein solubility

Question 33

what is the metabisulfate test used for

Answer 33

sickle cell

-dec pH causes sickling

Question 34

HbC defect

Answer 34

substitution of glutamate for lysine

Question 35

what is the defect in PNH

Answer 35

impaired synthesis of GPI anchor

mutation in phosphotidylinositol glycan A

Question 36

how can you Dx PNH

Answer 36

Hams test

flocytometry