Heme/Onco III Flashcards Preview

Board Review - C - Heme/Oncology > Heme/Onco III > Flashcards

Flashcards in Heme/Onco III Deck (35)
1

eosinopenia

cushing
corticosteroids

2

corticosteroid

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

3

porphyria

defect heme synthesis - accumulation of precursors

4

lead inhibit what enzymes

ferrochelatase (mito)
ALA dehydratase (cytoplasm)

5

acute intermittent porphyria

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

6

rate limiting step heme synthesis

D-ALA synthase
-cofactor B6

in mitochondria

D-ALA acid then to cytoplasm

7

porphyria cutanea tarda

uroporphyrinogen decarboxylase deficient
-cytoplasm

accumulation - uroporphyrin - tea colored urine

blostering cutaneous photosensitivity

MC porphyria

8

iron poisoning

peroxidation of membrane lipids

chelate - deferoxamine and dererasirox (oral)

9

PT test

extrinsic pathway
1, 2, 5, 7, 10

10

PTT test

intrinsic pathway
all factors - except 7 and 13

11

hemo A

X - linked recessive

factor 8

12

hemo B

X-linked recessive

factor 9

13

hem C

X-linked recessive

factor 11

14

hemarthroses, bleeding after trauma

hemophilia

15

tx hemophilia

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

16

vit K deficient

coag defect - normal bleeding time

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

17

platelet disorder

mucous membrane bleeds, epistaxis, petechiae

long bleeding time

18

bernard soulier

Gp Ib

defect platelet plug formation

19

glanzmamm

Gp IIb/IIIa

defect plateletaggregation

20

immune thrombocytopenia

anti GpIIb/IIIa Abs

splenic macro consumption of Abs

with viral illness

see increased megakaryocytes
tx - steroid - IVIG

21

thrombotic thrombocytopenic purpura

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

22

pentad of TTP

neuro and renal sx
fever
thrombocytopenia
microangiopathc hemolytic anemia

23

vWD

decreased vWF
-carrier of factor VIII - see PTT longer

and long bleeding time - platelet defect

24

tx vWD

desmopressin

25

DIC

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

26

antithrombin deficiency

no increase PTT after heparin admin

hypercoag

27

factor V leiden

mutant factor 5 resistant to protein C inactivation

hypercoag

28

protein C and S deficiency

no inactivation factor 5 and 8

more thrombotic skin necrosis

29

tissue necrosis after warfarin

protein C or S deficiency

30

prothrombin gene mutation

3' untranslated region

more prothrombin

higher plasma levels - and venous clots

31

packed RBC

Hb and O2 carrying capacity

32

platelets

5000 increase per unit

33

fresh frozen plasma

clotting factors

warfarin reversal, DIC, cirrhosis

34

cryoprecipitate

fibrinogen
factor 8
factor 13
vWF
fibronectin

tx coag factor deficiency - fibrinogen or factor 8

35

citrate

Ca chelator