Heme/Onco III Flashcards Preview

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

Flashcards in Heme/Onco III Deck (35)
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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