Heme/Onco III

Question 1

eosinopenia

Answer 1

cushing

corticosteroids

Question 2

corticosteroid

Answer 2

decreased activation of neutrophil adhesion molecule

impair migration out of vasc

sequester eosinophil in lympho node - cause to die

high neutro
low eosino and lympho

Question 3

porphyria

Answer 3

defect heme synthesis - accumulation of precursors

Question 4

lead inhibit what enzymes

Answer 4

ferrochelatase (mito)
ALA dehydratase (cytoplasm)

Question 5

acute intermittent porphyria

Answer 5

porphobilinogen deaminase deficient
-cytoplasm enzyme

accumulation - porphobilinogen, d-ALA, coporphobilinogen

pain abdomen
port wine urine
polyneuropathy
psych disturbance
precipitated by drugs, alcohol, staration

tx - glucose and heme - inhibit ALA synthase

Question 6

rate limiting step heme synthesis

Answer 6

D-ALA synthase
-cofactor B6

in mitochondria

D-ALA acid then to cytoplasm

Question 7

porphyria cutanea tarda

Answer 7

uroporphyrinogen decarboxylase deficient
-cytoplasm

accumulation - uroporphyrin - tea colored urine

blostering cutaneous photosensitivity

MC porphyria

Question 8

iron poisoning

Answer 8

peroxidation of membrane lipids

chelate - deferoxamine and dererasirox (oral)

Question 9

PT test

Answer 9

extrinsic pathway

1, 2, 5, 7, 10

Question 10

PTT test

Answer 10

intrinsic pathway

all factors - except 7 and 13

Question 11

hemo A

Answer 11

X - linked recessive

factor 8

Question 12

hemo B

Answer 12

X-linked recessive

factor 9

Question 13

hem C

Answer 13

X-linked recessive

factor 11

Question 14

hemarthroses, bleeding after trauma

Answer 14

hemophilia

Question 15

tx hemophilia

Answer 15

desmopressin and factor VIII - A
factor IX - B
factor XI - C

Question 16

vit K deficient

Answer 16

coag defect - normal bleeding time

decreased 2, 7, 9, 10 - protein C and S

Question 17

platelet disorder

Answer 17

mucous membrane bleeds, epistaxis, petechiae

long bleeding time

Question 18

bernard soulier

Answer 18

Gp Ib

defect platelet plug formation

Question 19

glanzmamm

Answer 19

Gp IIb/IIIa

defect plateletaggregation

Question 20

immune thrombocytopenia

Answer 20

anti GpIIb/IIIa Abs

splenic macro consumption of Abs

with viral illness

see increased megakaryocytes
tx - steroid - IVIG

Question 21

thrombotic thrombocytopenic purpura

Answer 21

inhibition of ADAM TS13 - vWF metalloprotease

decreased degradation vWF multimers

• get platelet adhesion and aggregation
• leads to thrombosis

hemolysis - schistocytes and elevated LDH

tx - plasmapharesis - steroids

Question 22

pentad of TTP

Answer 22

neuro and renal sx
fever
thrombocytopenia
microangiopathc hemolytic anemia

Question 23

vWD

Answer 23

decreased vWF
-carrier of factor VIII - see PTT longer

and long bleeding time - platelet defect

Question 24

tx vWD

Answer 24

desmopressin

Question 25

DIC

Answer 25

widespread activation of clotting -get deficient clot factors - bleed out due to sepsis, trauma, obstetric complication, acute pancreatitis, malignancy, nephrotic syndrome, transfusion see D dimer, schistocytes, fibrinogen, factor V and VIII low

Question 26

antithrombin deficiency

Answer 26

no increase PTT after heparin admin hypercoag

Question 27

factor V leiden

Answer 27

mutant factor 5 resistant to protein C inactivation hypercoag

Question 28

protein C and S deficiency

Answer 28

no inactivation factor 5 and 8 more thrombotic skin necrosis

Question 29

tissue necrosis after warfarin

Answer 29

protein C or S deficiency

Question 30

prothrombin gene mutation

Answer 30

3' untranslated region more prothrombin higher plasma levels - and venous clots

Question 31

packed RBC

Answer 31

Hb and O2 carrying capacity

Question 32

platelets

Answer 32

5000 increase per unit

Question 33

fresh frozen plasma

Answer 33

clotting factors warfarin reversal, DIC, cirrhosis

Question 34

cryoprecipitate

Answer 34

``` fibrinogen factor 8 factor 13 vWF fibronectin ``` tx coag factor deficiency - fibrinogen or factor 8

Question 35

citrate

Answer 35

Ca chelator